Clin Rheumatol (2005) 24: 155–157 DOI 10.1007/s10067-004-0986-4
CASE REPORT
Ahmet Mesut Onat Æ Levent O¨zc¸akar Æ Kemal U¨reten _ Sedat Kiraz Æ Ihsan Ertenli Æ Meral C¸algu¨neri
Avascular necrosis of the femoral head foreshadowing familial Mediterranean fever: apropos of three cases
Received: 19 March 2004 / Accepted: 4 June 2004 / Published online: 4 September 2004 Ó Clinical Rheumatology 2004
Abstract We reported here on three patients in whom the diagnosis of familial Mediterranean fever was established after avascular necrosis of the femoral head had been detected. The pathogenesis and the management of this rare concomitance are discussed in the light of the relevant literature. Keywords Arthritis Æ Avascular necrosis Æ Familial Mediterranean fever Æ Hip
Introduction Familial Mediterranean fever (FMF) is a hereditary disease characterized by recurrent episodes of fever and serositis, i.e., peritonitis, pleuritis, and synovitis. It mainly affects people of Mediterranean stock. Arthritic manifestations are protean and although acute self-limited attacks predominate, chronic destructive joint disease can occur. In this report we describe three patients with avascular necrosis of the femoral head preceding the diagnosis of FMF.
Case reports Case 1 A 20-year-old young man was seen for his complaints of left hip pain for the last 1–2 years. He described the pain to have occurred for 15-day episodes every month. He _ Ertenli Æ M. C¸algu¨neri A. M. Onat Æ K. U¨reten Æ S. Kiraz Æ I. Department of Rheumatology, Hacettepe University Medical School, Ankara, Turkey L. O¨zc¸akar (&) Department of Physical Medicine and Rehabilitation, Hacettepe University Medical School, Ankara, Turkey E-mail:
[email protected] Tel.: +90-312-3094142 Fax: +90-312-3105769
declared that abdominal pain attacks lasting for 2–3 days had also accompanied the painful episodes after the third attack. His medical history was otherwise noncontributory. The physical examination was consistent with painful and limited motions of the left hip joint with normal neurological findings. Laboratory analysis was unremarkable. The X-rays of the left hip joint were suggestive of avascular necrosis of the femoral head (Fig. 1). Magnetic resonance imaging (MRI) scans confirmed the likely diagnosis (Fig. 2). As the patient’s complaints had resolved with colchicine treatment (1.5 mg/day) and the genetic analysis revealed MEFV gene positivity, he was diagnosed with FMF. He was later operated on whereby femoral core decompression was performed. He has not experienced any more attacks under colchicine therapy. Case 2 A 25-year-old young man was examined for recurrent pain episodes in the left hip joint for the preceding 6 years. Although his sole complaint had been left hip pain in the first 3 years, he added that recurrent episodes of abdominal pain and fever had also started to take place (two to three times a year) in the last 3 years. The medical history was otherwise unremarkable. His physical examination was relevant with painful and limited motions of the left hip joint. Laboratory analysis disclosed increased levels of fibrinogen, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR). Radiographs and MRI of the hip joint depicted avascular necrosis of the femoral head. He was diagnosed with FMF and colchicine treatment (2 mg/day) was commenced. The attacks had subsided during his followup and he refused the suggested orthopedic intervention. Case 3 A 33-year-old man with similar complaints was called for examination after his brother (case 2) had been
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Discussion
Fig. 1 Radiography of the patient (case 1) demonstrating the joint space narrowing and sclerosis in the left hip with a local radiolucent area of the femoral head
diagnosed with FMF. He had suffered from recurrent episodes of left hip pain for 14 years (bimonthly). Three years after the onset of hip pain, recurrent abdominal pain and fever attacks had started. He mentioned that he had received a likely diagnosis of FMF 5 years ago and that he had been on irregular colchicine treatment since then. His physical examination revealed limitations in the left hip joint motions. Acute phase reactants (fibrinogen, ESR, CRP) were found to be elevated. Radiological findings were consistent with avascular necrosis of the left femoral head. He was given colchicine treatment (2 mg/day), and he has been followed up and remained free of any attacks. The hip pathology could only be followed conservatively as he also refused a surgical approach. The overall number of FMF patients that have been followed up in our department is about 250–300 and none of our three patients had any risk factors for avascular necrosis of the femoral head such as alcohol, steroid therapy, trauma etc.
Fig. 2 Magnetic resonance imaging of the left hip joint (case 1). a T1-weighted sagittal view. b T2-weighted sagittal view. c T2-weighted transverse view clearly demonstrating the necrosis of the femoral head
Arthritis in FMF can occur and by far comprises acute attacks with complete resolution within a few days or 2 weeks. The attacks may rarely assume protracted courses lasting several months and overall, chronic joint disease has been estimated to contribute fewer than 5% of the joint manifestations in FMF [1]. The involvement of knee, ankle, hip, and elbow joints is preponderant [2]. The underlying pathogenesis is usually a nonspecific synovitis whereby the small vessels of the synovial membrane have been postulated to be the principle target [3, 4]. Diminished chemotaxis inhibition in the synovial fluids of FMF patients has also been considered to play a role [5]. The hip is the most vulnerable site affected by the protracted attacks that may result in destruction of the articular cartilage and, less likely, avascular necrosis of the femoral head [2]. The latter has been mentioned in quite a few FMF patients in the literature; in this report we describe an additional three FMF patients with avascular necrosis of the femoral head. Interestingly, in our cases the hip pathologies did herald their underlying FMF. It is also noteworthy that the interim between the onset of hip arthritis and the typical abdominal attacks had been quite variable (3 months–3 years) in our patients. In a review of 1140 FMF patients, Sneh et al. [6] reported that only 57 (5%) individuals experienced protracted attacks involving 84 joints, 36 knees and 25 hips. Functional and, usually, anatomical integrity was regained in all but 27 joints. Of the 27 joints producing residual incapacity, 21 were hips. Seven of them showed roentgenologically typical avascular necrosis of the femoral head and 14 only sclerosis and narrowing of the joint space. Overall, eight hips eventually required total prosthetic replacement. They have suggested that the poor prognosis of the hip, in contrast to other joints affected by the protracted FMF arthritis, would be related not directly to the metabolic aberration underlying the disease but to attenuation of the arterial blood supply of the femoral head by synovial exudation [6]. This may be due to either increased capsular tension or the involvement of the small vessels of the synovial membrane [4]. Accordingly, it has also been proposed
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that early aspiration of the exudate might be beneficial in preventing the necrosis of the femoral head [6]. Once degenerative or necrotic changes ensue, the recurrent attacks of arthritis are no longer punctuated by symptom-free intervals in FMF. Besides, the irreversible morphological changes in the joints become irresponsive to colchicine or nonsteroidal anti-inflammatory treatment. Although ruling out a coexisting chronic inflammatory arthritis should remain a prerequisite [7], surgery is warranted in such cases [8, 9]. Overall, presenting three rare cases of femoral avascular necrosis, we have drawn attention to this untoward eventuality which might even be a harbinger of FMF. Besides orienting clinicians towards being vigilant against this adversity, we also imply that FMF should be included in the roster of differential diagnosis for avascular necrosis of the femoral head in patients with an as yet undetected underlying pathology.
Take home message Arthritis in FMF rarely assumes a protracted course lasting several months and the hip joint is the most vulnerable site affected by these attacks. They may result in avascular necrosis of the femoral head, which might even be a harbinger of FMF. We imply that FMF should also be included in the roster of differential diagnosis for avascular necrosis of the femoral head,
especially in patients in whom involvement of the hip joint displays a recurrent pattern.
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