Aug 22, 2018 - Background: Transverse myelitis (TM) refers to inflammation across ... inflammatory disorders such as systemic lupus erythematosus (SLE).
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episode or excessive activity. Different experiences of pain and discomfort included; articular pain, aching muscles, headaches, peripheral paresthesia, noise intolerance, bowel discomfort, nausea, eye discomfort, cramp-like pain in the parotid glands and pain/ discomfort relating to ill-fitting dentures and dental treatments. These experiences sometimes prevented engagement in social/leisure activities and could contribute to social isolation. Patients believed that when others - including family and clinicians - demonstrate little understanding of these symptoms, it had a negative impact on their mood. Self-management strategies used by patients included regularly setting an achievable and enjoyable goal, use of TENs devices, and accessing alternative therapies e.g. Shiatsu and acupuncture. Conclusion: Pain/discomfort in PSS is a heterogeneous concept. An empathic approach from the clinician towards patients’ pain/ discomfort may avoid further emotional distress for the patient. Experiences of pain and discomfort in PSS are unique and should be explored with the patient prior to planning appropriate targeted interventions. Disclosure statement: The authors have declared no conflicts of interest. 310. ORGANIZING PNEUMONIA AS A PRESENTING ¨ GREN’S SYNDROME FEATURE OF SJO Iman Ali1, Tom Sheeran1, Ahmed Fahim2 and Kirti Gupta3 Rheumatology, 2Respiratory and 3Endocrinology, Royal Wolverhampton Hospital, Wolverhampton, UNITED KINGDOM
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311. TRANSVERSE MYELITIS WITH ANTI-RO ANTIBODIES AS A PREDICTOR OF RECURRENCE Salema Khalid and Cecil B. Colaco Rheumatology, Central Middlesex Hospital, London, UNITED KINGDOM Background: Transverse myelitis (TM) refers to inflammation across the width of the spinal cord. The three main categories in the differential diagnosis of TM are demyelination, viral infections and inflammatory disorders such as systemic lupus erythematosus (SLE) and Sjogren’s syndrome. While TM is usually monophasic, there have also been cases of recurrent TM in association with anti-Ro antibodies without clinical connective disease manifestations. Methods: The association of TM and this autoantibody is suggestive of an autoimmune aetiology underlying the CNS inflammation. The presence of these antibodies is known to cause damage to cardiac tissue further adding weight to the autoimmune aetiology suggested above. We describe three cases of TM associated with anti-Ro autoantibodies without connective tissue disease manifestations and discuss the potential mechanisms behind the CNS insult. We also discuss several possible clinical and pathologic implications of the association of antiRo antibodies and TM, both as a predictor of recurrence as well as potential treatment strategies using immunosuppression. Results: All 3 cases reported were positive for Anti-Ro antibodies and had recurrent TM. Conclusion: The presence of Anti-Ro antibodies in patients with TM is a predictor of recurrence and has potential therapeutic implications with a strategy of using immunosuppression both in the acute and maintenance phases of treatment. Disclosure statement: The authors have declared no conflicts of interest.
Background: Pulmonary involvement is common in primary Sjogren’s syndrome (PSS) and approximately 10 to 20 percent of patients develop significant respiratory disease. Methods: We present a case of organizing pneumonia (OP) where the diagnosis of primary Sjogren’s syndrome followed the presentation of a young male with suspected Cryptogenic Organizing Pneumonia. Results: 27 year old gentleman with no significant past medical history. He initially presented with symptoms of chronic cough and pleuritic chest pain for 6 months. He was treated for community acquired pneumonia without resolution of bilateral apical opacities on chest radiograph. HRCT of thorax demonstrated apical consolidative changes with scattered inflammatory nodules. Ultrasound guided biopsy of parenchymal lung opacity showed evidence of OP with no associated conditions on detailed immunological work up. He was commenced on oral corticosteroids for COP leading to a remarkable initial improvement before subsequently deteriorating and presenting with bilateral painful swelling of the salivary and mandibular glands, significant dryness in his mouth, myalgia, generalized joint pain and considerable weight loss. Initial ultrasonic assessment of the parotid glands revealed abnormal non-specific features and subsequent biopsy was consistent with histological features of PSS. IgG subclass and IgG4 staining of parotid biopsy ruled out IgG4 disease (Table 1). Investigations for lymphoma and infective causes were negative. His symptoms settled down with 3g of IV methylprednisolone over 3 days. He was subsequently started on azathioprine and oral prednisolone. This patient is currently under the joint care of rheumatology and respiratory physicians in order to optimize his management and closely monitor for disease complications.
Background: It is recognized that CD4þ T helper (Th) cells play a crucial role in the pathogenesis of primary Sjo¨gren’s syndrome (pSS). Previous studies identified a predominance of Th1 phenotype, in particular in pSS patients with increased histological scores and presence of serum autoantibodies. We aimed to identify the peripheral T-cell phenotype abnormalities in patients with pSS compared to secondary SS associated with systemic lupus erythematosus (SS/SLE) and SLE alone, and correlate them with clinical and serological parameters, and lipid raft expression as marker of T cell activation. Our hypothesis is that disturbance in the functional properties of peripheral blood T cells can contribute to the immunopathogenesis of SS, and could have therapeutic potential. Methods: Blood samples and clinical and laboratory parameters from 32 patients with pSS, SLE and SS/SLE and 13 age/sex matched HC were obtained. We used flow-cytometry to perform T-cell immunophenotyping and analysis of lipid-raft expression. Results: Comparisons between different T cell population expressions (NS - no significant difference) are summarized in Table 1.
310 TABLE 1 Test results
Disease/T cell populations
312. INCREASED EXPRESSION OF LIPID RAFTS AS A MARKER OF CD4þ T CELL ACTIVATION CORRELATED WITH DISEASE ACTIVITY SCORE IN PATIENTS WITH PRIMARY ¨ GREN’S SYNDROME SJO Nicolyn L. Thompson, Akash Gandhi, Rebecca Radmore, Su Cho, Vivek Gupta, David Isenberg, Elisabeth Jury and Coziana Ciurtin Department of Rheumatology, University College London, London, UNITED KINGDOM
312 TABLE 1 Comparisons between different T cell populations
IgG4 staining ANA Anti-dsDNA C3 Inflammatory markers
Normal Negative Negative 1.75 Raised
Conclusion: Organising Pneumonia is considered a rare ILD in the context of PSS and this case is unusual as the diagnosis of OP predated the diagnosis of PSS. This case highlights the challenges associated with the diagnosis particularly if it presents in an atypical fashion in a male without classical immunological picture. Organizing Pneumonia is commonly associated with rheumatoid arthritis, but is rarely reported in conjunction of PSS. We should consider this diagnosis in cases of COP where response to corticosteroids is sub-optimal and there are clinical features of parotid involvement. Disclosure statement: The authors have declared no conflicts of interest.
SLE vs. HC
SS/SLE vs. HC
NS NS
NS NS
NS NS
NS NS Increased (p ¼ 0.0603)
NS NS Increased (p ¼ 0.1562)
NS NS Increased (p ¼ 0.0082)
NS
Decreased (p ¼ 0.0875) Increased (p ¼ 0.0196) Decreased (p ¼ 0.1039) Increased (p ¼ 0.0196) Decreased (p ¼ 0.0266)
NS
þ
CD4 T cells Naı¨ve CD4þCD27CD45RAþ Effector memory CD4þCD27CD45RA Activated CD4þCD25þCD127þ Effector CD4þCD25-CD127 Regulatory CD4þCD25þCD127 CD8þ T cells Naı¨ve CD8þCD27CD45RAþ Effector memory CD8þCD27CD45RA Activated CD8þCD25þCD127 þ
NS NS
Effector CD8 CD25 CD127
NS
T responder cells CD8þCD25CD127þ
Decreased (p ¼ 0.6091)
NS NS NS NS
Total lipid raft expression in CD4þ T cells correlated positively with the disease activity score in pSS (ESSDAI) (p ¼ 0.0305, r ¼ 0.4617), while the global BILAG score correlated positively with CD4þCD25CD127þ T responder in SLE patients (p ¼ 0.0235, r ¼ 0.7026). NS, no significant difference.
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