Differential Diagnosis and Management of Ankylosing ... - jospt

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CHELSEA L. JORDAN, PT, DPT1 • DANIEL I. RHON, PT, DPT, DSc2

Differential Diagnosis and Management of Ankylosing Spondylitis Masked as Adhesive Capsulitis: A Resident’s Case Problem

L

ow back pain (LBP) is a primary symptom prompting patients with ankylosing spondylitis to seek healthcare.1,3,8,43,55 Although the prevalence of ankylosing spondylitis is low, affecting between 0.1% and 1.0% of the population,7,11,36 it represents 5% of all LBP-related visits7 and can be significantly more debilitating than the more common nonspecific LBP. Many patients experience a variety of symptoms related to ankylosing spondylitis for 7 to 10 years before TTSTUDY DESIGN: Resident’s case problem.

TTBACKGROUND: Ankylosing spondylitis is a

potentially debilitating seronegative spondyloarthropathy, with inflammatory low back pain as the most commonly reported symptom. In the absence of low back pain, identification of other diagnostic criteria or associated impairments and joint involvement, such as involvement of the hip or shoulder, may be beneficial.

TTDIAGNOSIS: A 32-year-old man with right

shoulder pain and decreased range of motion was referred with a diagnosis of adhesive capsulitis. He had been managed by multiple healthcare providers for 3 years before being referred to a physical therapist. Glenoid labral pathology was evident on prior magnetic resonance imaging, which had led to a persistent focus on the shoulder. The evaluation by the physical therapist revealed significant mobility deficits in the cervical, thoracic, and lumbar spine. Radiographs and laboratory tests were ordered and a referral was made to rheumatology after the initial physical therapy assessment. The

diagnostic work-up confirmed the diagnosis of ankylosing spondylitis and led to multidisciplinary management of the disease.

TTDISCUSSION: Low back pain is often the

primary symptom of ankylosing spondylitis later in the disease process. Earlier indicators of ankylosing spondylitis, such as severely impaired mobility and spine stiffness, may help guide detection in the absence of spinal pain. In this case, an appropriate diagnosis led to improvement in the management strategy of what might have appeared to be unrelated shoulder pain. Early differential diagnosis is important, as emerging interventions show promise when used earlier in the disease process.

TTLEVEL OF EVIDENCE: Differential diagnosis,

level 4. J Orthop Sports Phys Ther 2012; 42(10):842-852, Epub 26 July 2012. doi:10.2519/ jospt.2012.4050

TTKEY WORDS: low back pain, physical therapy, sacroiliac joint, spondyloarthropathy

a correct diagnosis is made.18 Often, these may be reported in regions of the body other than the spine, such as the hip or shoulder. Ankylosing spondylitis is primarily a spine disease. Its most typical characteristic is inflammatory LBP, in which multiple articular joint surfaces of the spine are affected. Enthesitis, which is inflammation of the site where a ligament, tendon, or joint capsule attaches to bone, often results in new bone growth (syndesmophytes) and can be responsible for the inflammatory pain. A spinal disc may even be replaced by new bone during this process, which can result in fusion of the joints (ankylosis), accounting for the often-associated severe mobility restrictions, pain, and stiffness.22 However, inflammatory LBP is not always the earliest symptom of ankylosing spondylitis and therefore may not be the primary reason for seeking care,31,47 thus delaying the diagnosis, as shown in the patient of this resident’s case problem. Other signs and symptoms of the disease that may be revealed during the physical examination include peripheral joint enthesitis, uveitis, dactylitis, psoriasis, inflammatory bowel disease, and arthritis of the

Physical Therapist, 4th Stryker Brigade Combat Team, 2nd Infantry Division, Fort Lewis, WA. 2OIC, Department of Physical Medicine & Rehabilitation Service, Madigan Army Medical Center, Tacoma, WA. Institutional Review Board approval was not required for this case report. Publication of this case study was approved by the Department of Clinical Investigation at Madigan Army Medical Center. The views expressed are those of the authors and do not reflect the official policy of the Department of the Army, the Department of Defense, or the US Government. Address correspondence to Captain Chelsea L. Jordan, Madigan Army Medical Center, Department of Physical Medicine & Rehabilitation Service—Physical Therapy Section, Building 9040, Fitzsimmons Drive, Tacoma, WA 98431. E-mail: [email protected] t Copyright ©2012 Journal of Orthopaedic & Sports Physical Therapy 1

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FIGURE 1. Body chart: location and description of patient’s symptoms at the time of initial evaluation by the physical therapist. Note that the spine is cleared as a “pain-free” area. Abbreviations: I, intermittent; P1, pain 1 (primary reason for seeking care); P2, pain 2; V, variable.

peripheral joints.39 Decreased mobility from the ankylosing process and progressive deformity of the spine can affect motion and function of peripheral joints, especially those directly adjacent to the spine (hip, shoulders, and potentially knees),22 leading to significant pain and disability. Of these joints, the shoulder appears to be the most commonly involved (7%-58% of cases),17,57 and the hip is more often involved in those who have severe shoulder involvement.21 The duration of the disease may be the best indicator of shoulder involvement.17,57 Shoulder involvement in patients with ankylosing spondylitis has also been linked to worse spinal mobility and decreased quality of life.17 There is limited evidence indicating that women with ankylosing spondylitis may be more likely to have severe shoulder pain and stiffness than men.57 Peripheral joint symptoms should be investigated thoroughly for their potential association with other systemic disease processes. The purpose of this resident’s case problem is to describe the differential diagnosis process and treatment pathway

for the management of a patient initially referred for adhesive capsulitis, with an ensuing diagnosis of ankylosing spondylitis. The secondary purpose is to provide a review of the literature for diagnosis and classification of ankylosing spondylitis in the context of the multidisciplinary management of this patient.

DIAGNOSIS

A

32-year-old Caucasian man (height, 1.8 m; weight, 89.1 kg; body mass index, 27.4 kg/m2) was referred from his primary care physician in family medicine to an outpatient physical therapy clinic with the diagnosis of adhesive capsulitis and a request to “evaluate and treat.” This case met the subject rights protection criteria and was cleared for publication by the Department of Clinical Investigation at Madigan Army Medical Center.

Initial Presentation and History The patient’s primary reason for seeking care was a deep, sharp pain in the right shoulder, as well as decreased mo-

tion in the same shoulder. The pain was localized to the anterior aspect of the right glenohumeral joint and extended into the lateral aspect of the shoulder, arm, and forearm (FIGURE 1) only when exacerbated by throwing activities. He denied having any paresthesias in the upper extremity. His pain, as reported with a numeric pain rating scale, ranged from 0/10 at best to 9/10 at worst. The activities that aggravated his symptoms included throwing a ball and performing push-ups. Because the patient was a military service member, push-ups were a functional work-related task performed on a regular basis. However, the patient also participated in recreational activities that required overhead throwing, and returning to these activities was very important to him. He also reported an increase in pain with cold or rainy weather and the presence of occasional mild morning stiffness, localized only to the shoulder, with unreported duration. The patient reported a decrease in symptoms with rest, heat, and medication (325 mg of acetaminophen as needed). The patient denied having any significant nocturnal symptoms or pain anywhere in the spine (cervical, thoracic, or lumbar). His current shoulder symptoms had been present for approximately 3 years, and there was no apparent mechanism of injury that he could recall. He attributed the shoulder pain to overuse during basic training in the military. The patient worked in a cafeteria, and daily activities included walking and standing for long periods (in the cafeteria) and carrying and lifting items (occasionally overhead). He did not report any difficulty with these activities.

History of Treatment In the 3 years prior to the evaluation by the physical therapist, the patient had been referred to a chiropractor and an orthopaedic physician by his primary care provider. The patient had seen an orthopaedic physician on a total of 4 occasions, focusing on his shoulder hypo-

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mobility and pain. He was given a home exercise program consisting primarily of posterior glenohumeral joint capsule stretching and shoulder-strengthening exercises. The patient reported compliance with this program 3 to 5 times per week, without significant improvement of shoulder symptoms. The patient also saw a chiropractor over a 10-month period for decreased range of motion and pain in the cervical spine. This care consisted of 21 sessions that included a home exercise program, heat modalities, joint manipulation (to the cervical spine, pelvis, and rib cage), and electrical stimulation modalities to the right shoulder and cervical spine. At the end of this treatment period, reduced range of motion was still present, but neck pain was not reported by the patient. At this time, the patient was referred to see a physical therapist from family medicine and was given a diagnosis of adhesive capsulitis and a request to “evaluate and treat.” It was uncertain whether the previous referrals to the chiropractor and orthopaedic physician came from the same primary care provider. Six months prior to this evaluation, shortly after his return from military deployment, the patient had an insidious onset of hip pain, which was diagnosed as trochanteric bursitis. Physical therapy for his hip consisted of 10 sessions, including stretching exercises, pain modalities, and functional hip-strengthening exercises provided over a 3-month period. The patient was discharged after resolution of hip pain and meeting his discharge goals (greater than 73/80 on the Lower Extremity Functional Scale). During that episode of care, which ended 3 months prior to the current evaluation of his shoulder, the patient made no mention of low back or shoulder pain.

Systems Review The patient denied sudden weight changes, headaches, night pain, fever, chills, night sweats, or dizziness. He also denied any recent trauma or known mechanism of injury. His symptoms were provoked

with activity (throwing), which was consistent with the initial hypothesis of pain of musculoskeletal origin. The patient’s family history included diabetes mellitus (type 2) on his paternal side. The patient denied a personal or family history of alcohol abuse. The patient reported tobacco use of less than 1 pack per year and no alcohol use. The patient also denied any history of prior shoulder injury or surgery to the spine or extremities. Diagnostic imaging taken prior to the physical therapy evaluation included radiographs of both shoulders, which indicated subarticular cystic changes of the humeral head, and an arthrogram of the right shoulder revealing a Hill-Sachs lesion, a posterior/inferior labral tear, and a partial infraspinatus tear. Some of these injuries might have been related to his recreational overhead throwing activities. There was no record of lumbar or cervical spine imaging, nor were there records of laboratory tests having been performed.

Physical Examination Observation Visual assessment of the

patient’s gait when walking from the waiting room to the examination room showed a lack of isolated segmental movement between the lumbar spine and pelvis. His arm swing during gait appeared to be decreased, suggesting potential loss of shoulder mobility. Decreased cervical spine mobility limited his ability to lie supine and rest his head back on the treatment table. Similar limitations in his lumbar spine restricted him from performing a sit-up or properly moving from a supine to a sitting position. He was forced to log roll to get up from a reclined position and had to rock his pelvis to transition from a reclined to a seated position. Mobility Testing Considerable limitations were found with active and passive motion testing of the shoulders, cervical spine, thoracic spine, and lumbar spine. Though there was loss in all planes of spinal motion, this loss was most apparent in extension of the lumbar spine (exten-

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sion to neutral) and extension and sidebending of the cervical spine, with only 5° of available motion in each plane. Although the patient perceived only having significant right shoulder range-of-motion deficits, he actually had significant limitations bilaterally. The patient was limited to 125° of shoulder passive range of motion bilaterally. In passive shoulder abduction, the patient had 112° of abduction on the left and 95° on the right. In addition, accessory joint movement assessment of the lumbar and cervical vertebrae revealed relatively little mobility with attempted lateral glides and posterior/anterior joint mobilizations. Thoracic joint mobility was limited as well. None of the mobility assessment testing reproduced his symptoms. Strength Testing Minimal strength deficits were noted on the right compared to the left side in shoulder external rotation (4/5), internal rotation (4+/5), and abduction (4+/5). Shoulder adduction strength was symmetrical. The patient denied pain with muscle strength testing. Special Tests The following diagnostic physical examination tests, performed to assess for impingement and rotator cuff tendinopathy, were negative: Neer impingement sign, Hawkins sign, pain provocation, teres minor sag sign, and subscapularis lift-off tests. In the cervical spine, the Spurling test (foraminal compression test) was performed, which reproduced some mild cervical pain but no peripheralization or reproduction of symptoms in the shoulder or upper extremity. Neurological Testing A neurological examination was performed secondary to the reports of pain extending down into his arm and wrist with the motion of throwing. The myotatic stretch reflexes, myotomes, and dermatomal testing for light touch sensation were all normal bilaterally for C5, C6, and C7 nerve roots. Additionally, the patient denied any numbness or tingling in either upper extremity. Upper motor neuron testing and lower extremity neurological testing were deferred at this time.

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FIGURE 2. Radiographs showing bilateral, symmetric, severe narrowing of the sacroiliac joints.

Interpretation of the History and Physical Examination The impairments identified at the shoulder included pain, range-of-motion limitations, and muscle weakness. The combination of signs and symptoms indicated a need for further screening, including (1) bilateral mobility restrictions of the shoulders, (2) significant reduction of mobility of the entire spine, (3) age of the patient, (4) significant shoulder pathoanatomy identified with diagnostic imaging despite irrelevant history or mechanism of injury, (5) an examination initially unconvincing of a diagnosis of adhesive capsulitis, and (6) no improvement with 3 years of conservative management. The potentially distracting variable with this case was the absence of reported LBP.

Diagnosis Confirmation After considering the findings of the initial physical therapy evaluation, and based on the interpretation of the history and physical examination, the physical therapist suspected a systemic rheumatic disease process, such as psoriasis, reactive arthritis, or ankylosing spondylitis, and collaborated with the patient’s primary care provider to coordinate further evaluation for the patient. In the US military healthcare system, the primary care provider is typically a physician in family medicine who manages the overall healthcare of the patient. However, specialty services can refer between specialty

FIGURE 3. Radiographs showing partial/complete ankylosis at both sacroiliac joints and calcification within the pelvis.

services, and a physical therapist can refer directly to another specialty service if indicated. In this case, the physical therapist coordinated further care with the primary care provider based on what she thought was the best course of action to specifically rule out ankylosing spondylitis, as this was the diagnosis of most concern to the physical therapist. The following laboratory and imaging tests were ordered to help establish a diagnosis: human leukocyte antigen (HLA)-B27, C-reactive protein, erythrocyte sedimentation rate, and radiographic views of the cervical spine, thoracic spine, lumbar spine, and sacrum. A rheumatology consultation was also recommended. Results of the above laboratory tests indicated that the C-reactive protein

was high (3.2) and the rheumatoid factor and erythrocyte sedimentation rate were negative. The patient was subsequently evaluated by a rheumatologist, who also suspected a diagnosis of ankylosing spondylitis. The diagnostic process by the rheumatologist took several weeks, as various screening and confirmatory tests were ordered and interpreted. The diagnostic work-up included ordering additional laboratory tests (including the HLA-B27 gene expression, which was present) and interpretation of the radiographs. Ankylosing of the sacroiliac joints and spine was identified on the radiographs (FIGURES 2 and 3), which alone increases the odds ratio for diagnosis of ankylosing spondylitis to 32.3.39,42 Multiple tests were ordered because radio-

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Assessment of   SpondyloArthritis International   Society Classification Criteria 39*

TABLE 1

Imaging arm†‡

• Sacroiliitis on imaging plus 1 or more SpA features

HLA-B27 arm†

• Positive for HLA-B27 plus 2 or more SpA features

Criterion for sacroiliitis imaging arm (must have one or the other)

•A  ctive (acute) inflammation on MRI suggestive of sacroiliitis associated with SpA • Definite radiographic sacroiliitis according to the modified New York Criteria

SpA features

• • • • • • • • • • •

Inflammatory back pain Arthritis Enthesitis of the heel Uveitis Dactylitis Psoriasis Crohn’s disease/ulcerative colitis Good response to NSAIDs Family history of SpA Positive HLA-B27 Elevated C-reactive protein

Abbreviations: HLA-B27, human leukocyte antigen-B27; MRI, magnetic resonance imaging; NSAID, nonsteroidal anti-inflammatory drug; SpA, spondyloarthropathy. *Precursor to using classification criteria: 3 months or more of back pain, with onset of this pain before the age of 45 years. † Values for both the sacroiliitis imaging and HLA-B27 criteria have a sensitivity of 83%, a specificity of 84%, a positive likelihood ratio of 5.3, and a negative likelihood ratio of 0.20. ‡ Imaging arm alone: positive likelihood ratio, 20.7; negative likelihood ratio, 0.35.

TABLE 2

Criteria for Inflammatory Back Pain

Symptoms (Historical)40*

Symptoms (Expert Opinion)46†

Morning stiffness greater than 30 min

Improvement with exercise

Improvement with exercise but not with rest

No improvement with rest

Awaken during the second half of night due to back pain

Pain at night

Alternating buttock pain

Age at onset 40 y or younger

Inclusion criteria: less than 50 y of age and a minimum of 3 mo of low back pain

Insidious onset

2+ criteria: sensitivity, 70%; specificity, 81%; positive likelihood ratio, 3.7

4+ criteria: sensitivity, 77% and 80%; specificity, 92% and 72%; positive likelihood ratio, 9.63 and 2.86‡

3+ criteria: sensitivity, 33%; specificity, 98%; positive likelihood ratio, 12.4 *From clinical history of 213 patients with chronic back pain (101 with ankylosing spondylitis and 112 with mechanical low back pain). † From 13 rheumatologists in a workshop clinic, determining which factors were present in 20 patients with inflammatory back pain, followed by validation on 648 separate patients. ‡ The 2 values are the original and validation cohorts.

graphs alone should not be relied on to make the diagnosis. Additionally, in the absence of radiographic findings, which is often the case early in the disease process,18,55 the screening potential for ruling out ankylosing spondylitis is not

very strong (sensitivity, 19%; specificity, 47%).5 Scintigraphy has also been shown to be limited in its value for diagnosis and early detection of sacroiliitis, with a sensitivity of 51%.48 Magnetic resonance imaging (MRI) is better for ruling in sac-

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roiliitis, with a positive likelihood ratio of 9.0 (sensitivity, 90%; specificity, 90%)33,39 Though not always recommended as an initial test, it is also more valuable for screening, because normal MRI results in a negative likelihood ratio of 0.11. MRI and Doppler ultrasonography are better at detecting evidence of ankylosing spondylitis earlier in the disease progression, at a time when radiographs may still be negative.5 If radiographs are negative but the clinician still has a strong suspicion for the diagnosis, then MRI may be considered. A combination of multiple tests may provide the best diagnostic strategy. The Assessment of SpondyloArthritis international Society classification criteria are currently the best validated diagnostic criteria for the disease.39,47 If the patient is younger than 45 years of age and has back pain at least 3 months in duration, then evidence of ankylosis of the lumbopelvic spine on radiographs, combined with at least 1 spondyloarthropathy (SpA) feature47 (TABLE 1), has a positive likelihood ratio of 20.7 for the disease.47 Without imaging, the presence of the HLA-B27 gene and 2 SpA features in the same patient results in a positive likelihood ratio of 5.3.47 Appropriate laboratory tests include HLA-B27 gene expression, which is prevalent in approximately 90% to 95% of patients with ankylosing spondylitis.27,37 However, presence of the HLA-B27 gene alone should not be used as a general diagnostic tool, as it is present in many healthy, low-risk individuals and can lead to a false positive diagnosis.27,28 Only 5% of the population with HLA-B27 gene expression has ankylosing spondylitis; however, up to 95% of the patient population with the diagnosis of ankylosing spondylitis has the gene. 37 HLA-B27 has not been shown to specifically correlate with shoulder involvement in this patient population.17 This patient was found to be a carrier of the HLA-B27 antigen.

Course of Intervention During the diagnostic work-up by the rheumatologist, the physical therapist

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TABLE 3

Distinguishing Features for Differential Diagnosis

Associated Features

Ankylosing Spondylitis

Nonspecific LBP

Lumbar Spinal Stenosis

Rheumatoid Arthritis

Prevalence/incidence

0.01%-1.8%10,27,52,59

15%-45% of adults yearly, >70% within their lifetime (in developed nations)53

Acquired26: 4%-20% 60 y of age

1% of adults32

Age of onset

Teens to early adulthood (60 y of age26

40-50 y of age most common, but occurs earlier32

Disease duration

16.3 y20

Variable (acute to chronic)

Symptoms variable; condition remains indefinitely

Symptoms may vary, but progressive for life

HLA-B27 presence

90%-95%36

Not related

Not related

Poor association24

Male-female ratio

3:1

1:1

1:1

1:3

Night pain

Worse in last half of the night

Indifferent*

Indifferent*

Indifferent*

Response to NSAIDs

Excellent

Variable

Variable

Variable

Sacroiliitis frequency distribution

100% symmetric

Indifferent*

If present, usually bilateral (or symmetric)

Indifferent,* not a key feature

Peripheral arthritis, frequency distribution

Occasional, asymmetric

Not related

Not related

Common, especially upper extremity

Sacroiliitis (on imaging in general)

X† (common but not always early in disease)

Not related

Indifferent*

Not key feature

Definite radiographic sacroiliitis (grade 2 or higher bilateral or grade 3 or higher unilateral)

Odds ratio,‡ 32.3 (6.6, 166.7)39

Not related

Indifferent*

Not key feature

Unilateral grade 2 radiographic sacroiliitis

Odds ratio,‡ 7.8 (1.3, 50)39

Not related

Indifferent*

Not key feature

Active inflammation of the sacroiliac joints (MRI)

Odds ratio,‡ 40 (5.3, 383)14

Not related

Indifferent*

Not key feature

11,51

Table continued on page 848.

had the patient on a treatment plan that addressed the impairments found in the shoulder and spine. The supervised program included the following: supine and seated posture-based exercises, selfmobilizations for the thoracic spine, rotator cuff strengthening exercises, scapular stabilization and strengthening exercises, as well as cervical stretches. The patient was also treated with joint mobilizations (grades II-IV) to the glenohumeral joint and thoracic spine. The results from a systematic review suggest that for individuals with ankylosing spondylitis, both home-based and supervised exercise programs individualized to the patient are better than no intervention, and that supervised group physiotherapy is better than home exercises.15 A multimodal exercise approach that includes aerobic, stretching, and pulmonary exercises in addition to usual medical care may pro-

vide greater improvements in spinal mobility, work capacity, and chest expansion than usual medical care alone.23,34 In addition, manual therapy has been shown to be effective for improving spinal mobility in these patients.56 Based on the findings, the rheumatologist suspected the diagnosis of ankylosing spondylitis, and the patient was placed on the nonsteroidal anti-inflammatory drug (NSAID) naproxen (500 mg twice a day). NSAIDs are considered the first line of intervention for ankylosing spondylitis, and may even prevent new bone formation.9 In fact, a discriminating factor between ankylosing spondylitis and chronic LBP may be considerable relief of symptoms with NSAIDs within 48 hours.55 A good response to NSAIDs has been proposed as a useful criterion for the discrimination between axial SpA and no SpA, which also gives it some value in the

diagnosis for ankylosing spondylitis. 39 A poor response to NSAID treatment has also been correlated with poor prognosis for patients with ankylosing spondylitis.55 Two studies have shown that C-reactive protein levels in patients with ankylosing spondylitis were significantly decreased at 12 weeks with a regimen of diclofenac, naproxen, or celecoxib.4,44 This patient was able to gain satisfactory pain control with a naproxen regimen. Pharmacological treatment of inflammatory arthritides, such as ankylosing spondylitis and rheumatoid arthritis, includes not only a regimen of NSAIDs but also antitumor necrosis factor or biologic agents (also known as biological-response modifiers) and disease-modifying antirheumatic drugs. Each of these options differs in effectiveness based on the different pathogenesis of each disease. In ankylosing spondylitis, the pharma-

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TABLE 3

Distinguishing Features for Differential Diagnosis (continued)

Associated Features

Ankylosing Spondylitis

Nonspecific LBP

Lumbar Spinal Stenosis

Rheumatoid Arthritis

Morning stiffness

X† (>30 min)

Indifferent*

X†

X† (>1 h)2

Symmetric joint effusion

Common (sacroiliac only)

Not related

Indifferent*

X†

Rheumatoid nodules

Not related

Not related

Not related

X† (20%-30% of cases)13

Arthritis

Autoimmune: usually spine

Degenerative (OA)

Degenerative (OA)

Autoimmune: usually peripheral joints

Rheumatoid factor

Not present

Not present

Not present

X†

C-reactive protein

Sensitivity, 50%; specificity, 80%; positive likelihood ratio, 2.541

Not related

Not related

X†

Joint erosion (hands/feet)

Not common; spine and proximal Not related peripheral joints (shoulder/hip) are more common

Not related

X†

Diagnosed with an iritis

Odds ratio,‡ 30.31 (7.26, 126.49)55

Not related

Not related

Not common

Enthesitis

Sensitivity, 37%; specificity, 89%; positive likelihood ratio, 3.441

Not related

Not related

Not common

Dactylitis

Sensitivity, 18%; specificity, 96%; positive likelihood ratio, 4.541; odds ratio,‡ 5.3 (0.9, 29.4)39

Not related

Not related

Not related38

Uveitis

Sensitivity, 32%; specificity, 95%; positive likelihood ratio, 6.441

Not related

Not related

Most common with juvenile6

Crohn’s/colitis: IBD

AS symptoms present in 50% of IBD cases59; odds ratio,‡ 6.5 (0.6, 66.7)39

Not related

Not related

X†

Pain and/or stiffness in the hip

Odds ratio,‡ 3.54 (1.52, 8.23)55

Potential referral of pain to hip

Possible

Not common

Pain and/or stiffness in the neck

Odds ratio,‡ 3.49 (1.54, 7.94)55

Not related

Not key feature

Possible

Abbreviations: AS, ankylosing spondylitis; HLA-B27, human leukocyte antigen-B27; IBD, inflammatory bowel disease; LBP, low back pain; MRI, magnetic resonance imaging; NSAID; nonsteroidal anti-inflammatory drug; OA, osteoarthritis. *This condition has no tendency toward or away from this feature. † Association is present between this feature and the listed condition. ‡ With 95% confidence intervals when compared to chronic low back pain.

cological focus is often on the effect of the disease on bone regeneration, which is predominantly osteoproliferative.9 In contrast, rheumatoid arthritis tends to target the synovium and is osteodestructive to the cartilage of the body. 9 To reach and maintain a therapeutic level, a continuous dose of NSAIDs may be needed, but this is not always easy to recommend due to the gastrointestinal, renal, and cardiovascular risks associated with continuous NSAID use.50 In a cross-sectional study, more than 20% of patients with ankylosing spondylitis reported incomplete pain control with NSAID use, whereas over 40% required a change in medication due to lack of efficacy.58 The exact influence of NSAIDs on spondylitis and syndesmophyte forma-

tion needs further study. Although pain relief seems to be comparable between the commonly used NSAIDs naproxen, celecoxib, and diclofenac, celecoxib has fewer reported incidents of gastrointestinal complications.4,44 This patient also had other impairments associated with ankylosing spondylitis that prompted a referral to the pulmonary clinic, where he was diagnosed with restrictive lung disease due to the decreased chest wall expansion as a result of poor trunk mobility. This is not an uncommon finding in patients with ankylosing spondylitis, as the ankylosing process begins to hinder chest wall mobility.35 He was prescribed 40 mg of esomeprazole, fluticasone, and salmeterol. Esomeprazole is a proton-pump in-

hibitor that decreases production of acid in the stomach and is used to treat gastroesophageal reflux disease, which can be associated with pulmonary disorders, and to improve tolerance to NSAID use. Fluticasone and salmeterol are a steroid and bronchodilator, respectively, used in the treatment of obstructive pulmonary disorders, such as chronic obstructive pulmonary disease, by allowing the muscles in the airway to relax more. The manual therapy directed at the thoracic spine, the self-mobilizations, and the postural exercises were intended to help with these impairments.

Case Outcome The patient received care from the physical therapist for his shoulder symptoms

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for 7 sessions. Improvement in the patient’s symptoms and function was assessed using the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) scale, which has demonstrated good internal validity in other studies of this population.12 The BASDAI consists of 6 questions related to 5 aspects of ankylosing spondylitis, including (1) fatigue, (2) spinal pain, (3) joint pain/swelling, (4) areas of localized tenderness, and (5) morning stiffness (duration and intensity), using a 10-cm analog scale.12 The average of the 2 morning stiffness scores is taken and divided by 5 to give a final value between 0 and 10. Scores of 4 or greater suggest suboptimal control of the disease. There was no baseline measure for the patient, but his BASDAI score continued to improve after starting physical therapy (1.3, 0.9, and 0.3 at 1, 3, and 6 months, respectively), indicating improvement. The Modified Health Assessment Questionnaire scale is often used for assessing disability in patients with rheumatoid arthritis,49 but it is also used in patients with ankylosing spondylitis, because they often take similar drugs. It evaluates the patient’s functional disability with daily activities using 8 categories. Questions include topics of self-care, ambulation, and transportation. Scores of less than 0.3 are considered normal.49 The patient filled out the Modified Health Assessment Questionnaire for the rheumatologist nearly 1 year after seeing the physical therapist, and had a score of 0.5. Although we cannot compare the scores of the BASDAI to those of the Modified Health Assessment Questionnaire and adequately measure objective improvement, it appeared that, at a minimum, the patient was not getting any worse 1 year after the diagnosis was made. One year after the diagnosis, the patient switched from naproxen to sulindac (200 mg), which is a different class of NSAID, and also began taking glucosamine chondroitin, which is a dietary supplement thought to improve joint health. The decision to use more aggressive medi-

cal therapies was postponed due to a favorable response with sulindac alone.

DISCUSSION

I

n this resident’s case problem, we described various aspects of the assessment that contributed to the diagnosis of ankylosing spondylitis in a patient presenting primarily with right shoulder pain and stiffness and the subsequent management of the condition. Understanding the full spectrum of a systemic disease process is important to facilitate diagnosis in a timely manner, especially in cases where it can mimic other common musculoskeletal conditions such as shoulder pain. Ankylosing spondylitis has several common characteristics, of which inflammatory LBP is the most distinguishable. Without spine-related symptoms, however, a clinician may be misled and not consider a diagnosis of ankylosing spondylitis. In the present case, a significant time passed and a number of healthcare professionals were seen prior to diagnosis, illustrating that scenario. Prolonged stiffness and lack of mobility in the spine can adversely affect the mobility of adjacent peripheral joints. It is well documented that scapular dyskinesia and mobility are strongly associated with pathology and function in the shoulder.19,29,30 This alteration in the biomechanical dependence between adjacent joints may lead to excessive stress applied to the joint and associated structures, as might have been the case in the present patient, who had MRI-confirmed pathology of his right shoulder, including a Hill-Sachs lesion, labral tear, and partial tear of the infraspinatus. These imaging findings, combined with his exclusive shoulder symptoms, might also have led to a persistent focus on the right shoulder. The impaired mobility of the spine, the history of hip pain 3 months earlier, and the significant shoulder pathology without clear mechanism of injury in an otherwise healthy man collectively should have raised the suspicion for

an atypical presentation of a musculoskeletal condition or a potential systemic disease process. Additionally, there was a strong suspicion by the physical therapist that the clinical presentation did not match the previously given diagnosis of adhesive capsulitis, warranting further evaluation. Although this patient did not present with LBP, it is important to understand the relationship between LBP and ankylosing spondylitis. The prevalence of ankylosing spondylitis is low in comparison to nonspecific LBP (the fifth most common reason for all physician visits in the United States and 85% of all LBP-related visits),16,20,54 and this may be one of the reasons why it is not as easily identified. The presence of inflammatory LBP increases the probability of the diagnosis 3-fold in comparison to that of chronic, nonspecific LBP,41 and clinicians should be aware of key differentiating symptoms. However, it has been described as a significant challenge in primary care settings to differentiate between inflammatory LBP and mechanical LBP.25 Identifying the appropriate symptoms can generate a positive likelihood ratio as high as 12.440 and significantly improve the probability of correct differentiation (TABLE 2). Other differential diagnoses include rheumatoid arthritis, psoriasis, spinal stenosis, and sacroiliitis.40 Discriminators between ankylosing spondylitis and chronic LBP have been described in the literature, and a list of significant clinical variables is provided in TABLE 3.55 Multiple classification systems have also been derived to better differentiate a spondyloarthropathy from mechanical LBP. These are valuable in that their focus is not limited to signs and symptoms in the lumbar spine. The Assessment of SpondyloArthritis international Society has published classification criteria that have been validated and may prove to have the strongest classification value (TABLE 1).39 Some of these criteria still may not be sufficient in detecting those with early ankylosing spondylitis due to their dependence on the presence of inflammatory back pain, HLA-B27

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gene expression, or sacroiliitis on imaging studies. Although inflammatory back pain has historically been the hallmark symptom of ankylosing spondylitis, imaging changes associated with inflammatory LBP may not show up for up to 7 years,45 and there is evidence to suggest that these patients may not all initially present with complaints of LBP,31,45 as shown in this resident case. In addition, the classification guidelines do not address peripheral joint involvement, which might have been helpful with this case. Because the nature of the patient’s gait and obvious mobility impairments were inconsistent with a pathology limited to the shoulder, radiographs were ordered and used as one of several tools to gather more information about the patient’s condition. Other early indicators of disease in the absence of pain may include stiffness of the spine and complaints of pain in adjacent joints, such as the shoulder and the hip. In fact, this patient had been seen and treated by a physical therapist for a hip complaint 6 months earlier. At that time, the patient was diagnosed with trochanteric bursitis. The therapist documentation stated that “the patient moves with slow guarded movements and gait is mildly antalgic, but not ataxic,” and indicated clearing the lumbar spine and sacroiliac joints for any pathologies or complaints. After 3 months in physical therapy, the patient was discharged with a 2/10 on the numeric pain rating scale and a 73/80 on the Lower Extremity Functional Scale. In theory, his hip pain could have been an enthesitis masked as trochanteric bursitis, and a potential opportunity to further screen for ankylosing spondylitis might have been missed. There were no overt complaints consistent with arthritic origin identified on the documentation, although there were some hip hypomobility impairments noted from the physical examination. The therapist might not have used any tests in the assessment that were specific to ankylosing spondylitis. There are several methods reported to assess stiffness and spinal mobility specifically in this

patient population, with adequate levels of reliability and validity. These include the modified Schober index (15 cm),27 fingertip-to-floor distance,21 and trunkforward flexion.21 It is also important to note that the uncertainty of the diagnosis did not need to preclude treatment from the physical therapist. In the absence of red flags or suspicion for pathology requiring immediate medical intervention to favorably change the outcome or prognosis, the physical therapist was still able to identify functional impairments and formulate an intervention strategy to address these. This parallel thought process allowed the therapist to have a diagnostic management plan that included appropriate referrals and test procedures, while at the same time having a treatment management plan aimed at addressing significant impairments of spine and shoulder mobility and range of motion that had been identified. The prognosis and modifications to the treatment plan can be implemented and changed as more information about the specific diagnosis becomes available. Based on the initial information, there was no reason the physical therapist should not have initiated treatment, as determined to be within the clinician’s scope and clinical expertise to best manage the patient. The original diagnosis of the patient was questioned because it did not clearly match up with the current history and physical examination findings. Clinicians should continue to manage the patient until they are able to determine either that the patient does not belong in their clinic (after identification of a nonmusculoskeletal condition in the case of this physical therapist) or that the patient has met determined goals and/or maximized therapeutic potential under their care. This definitive decision may take a course of several visits, as further information is gathered, and does not always need to be made on the day of the initial evaluation. There should, however, always be a plan with a specific direction in place. This highlights the importance of reassess-

]

ment of the patient and consideration for alternate pathologies. Although this patient had no classic signs of ankylosing spondylitis, such as inflammatory back pain, his presentation, history, and overt spinal mobility limitations immediately raised suspicion, indicating the need for further screening.

CONCLUSION

T

his resident’s case problem described an atypical presentation of ankylosing spondylitis. Even with the initial absence of inflammatory LBP, the need for further screening was recognized based on abnormal movement patterns influenced by lack of spinal mobility. Inflammatory LBP is a symptom in patients with ankylosing spondylitis, manifesting well into the disease process, and is usually the catalyst for seeking healthcare. However, due to the high incidence of associated peripheral joint pathology, especially in the shoulder and hip, early complaints associated with the disease may initially present at these locations. In patients with hip and shoulder disorders that fail to resolve with conservative management and display obvious global limitations in spine and axial mobility, there may be indication to screen further for systemic inflammatory conditions, regardless of spine symptom presence. A physical therapist may not have the tools to confirm a systemic disease process, but should be able to identify factors that are not consistent with a normal musculoskeletal disease process and know when further testing and referrals are necessary. Ankylosing spondylitis is a relatively rare condition that can take as many as 10 years before being correctly diagnosed.55 Emerging treatments show promise of success when applied early in the disease process, highlighting the importance of an early diagnosis. t

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