fever (FMF) is an autosomal recessive disease in which arthritis attacks occur in about half of the patients, usually involving the ankle, knee, or hip (1,2). FMF arthritis is typically acute, episodic, and self-limited, resolving without sequelae; however, in ฯณ10%, it is protracted, lasting 3 months to 7 years (3,4). Colchicine might not be effective when started after the development of arthritis, and, in such cases, therapeutic alternatives remain unclear and some patients require synovectomy (4). Arthritis in FMF can also be destructive, most commonly affecting hip joints, and might necessitate hip replacement (5,6). Here we report a patient with FMF and protracted hip arthritis who had an early response to infliximab therapy.
A 21-year-old woman with a history of FMF presented with severe right hip pain and difficulty walking. FMF was diagnosed at age 3 years when she began to have attacks of fever, abdominal pain, and arthritis, usually affecting the knees and ankles, and was started on colchicine therapy. At age 14 years, indomethacin was added for arthritis attacks lasting 4 -5 days. Two years ago, colchicine and indomethacin were discontinued when she had an episode of bloody diarrhea that was self-limited. Biopsy of the duodenum was negative for amyloid. One week later, fever developed, followed by bilateral hip pain. An anteroposterior radiograph of the pelvis revealed significant joint space narrowing of the right hip (Figure 1). On magnetic resonance imaging (MRI) (Figure 2), coronal short inversion time inversion recovery images showed increased signal intensity consistent with bone marrow edema at the acetabulum bilaterally and at the lateral aspects of the left femoral head and neck. There was no evidence of joint effusion or synovial hypertrophy. Computerized tomography of the sacroiliac joints was normal. She was homozygote for M694V and was HLA-B27 negative. Colchicine 0.5 mg 3 times daily was restarted. Fever, abdominal pain, or diarrhea did not recur. However, right hip pain persisted, and 2 weeks later her followup erythrocyte sedimentation rate (ESR) was 110 mm/hour and the C-reactive protein (CRP) level was 96 mg/liter. Hip pain did not respond to nonsteroidal antiinflammatory drugs. Prednisolone 15 mg/day was started. A daily dose of 40 mg was required for pain control, which was accompanied by improvement in the ESR (13 mm/hour) and the CRP (6 mg/ liter). Tapering off of steroid therapy was unsuccessful. She was allergic to sulfasalazine. Hydroxychloroquine and methotrexate (up to 20 mg weekly) were added, but the