Table 3 Summary of case reports with overlap of familial Mediterranean fever and Behçet’s disease [17,18,19,20,21,22,23,24,25,26]
Citation | Patient | Symptomatology (duration) | Treatments | Outcomes |
|---|---|---|---|---|
Zerkaoui et al. [19] | 10F Origin: Morocco p.M693K | Periodic fever with abdominal pain, pseudo erysipelas, vomitus, monoarthritis, headache, one episode aphthous and urogenital ulcers (months), cerebral venous thrombosis (acute) | Colchicine intravenous Methylprednisolone × 3 days, followed by oral prednisone Enoxaparin | Intolerance to colchicine—response not determined Resolution of thrombus at 6 months Papillary edema with scleromalacia on ophthalmology follow-up |
Güler et al. [20] | 19F Origin: Not stated M694V/R202Q | Recurrent aphthous and genital ulcers, erythema nodosum (> 10 years) Recurrent attacks of fever, abdominal pain, arthralgia (2 years) | Colchicine 1.5 mg/day × 10 years (before admission) Colchicine 2 mg/day + methylprednisolone 16 mg/day, azathioprine 100 mg/day, diclofenac sodium 150 mg/day | Recurrent oral aphthae and genital ulcers despite colchicine 1.5 mg/day At 1 month follow-up on the new regimen, improved mucocutaneous ulcers, resolved acute phase reactants. Methylprednisolone gradually decreased to 8 mg/day. At 6 months follow-up, decreased severity and frequency of attacks |
Sunar et al. [21] | 43 M Origin: Not stated HLA-B51 positive | Aphthous stomatitis, inflammatory low back pain, arthralgias, febrile attacks with abdominal pain, genital ulcerations, erythema nodosum, sacroiliitis (> 8 years diagnosed FMF + spondylosing arthritis) | Sulfasalazine 2 g/day, colchicine 4 × 0.5 g and acemetacin 120 mg/day 8 years | Frequency of attacks decreased following colchicine treatment |
Mobini et al. [22] | 27 M Origin: Iran E148Q/P369S | Aphthous and genital ulcers, pustular folliculitis (5 years) Attacks of fever, chills, arthralgia, arthritis (5 months) | Colchicine (5 years) Added prednisolone and azathioprine during admission | Symptoms continued, azathioprine and colchicine discontinued due to new diarrhea and colchicine reintroduced when diarrhea resolved with symptom improvement |
Erdem et al. [23] | 23 M Origin: Not stated | Knee pain, swelling, abdominal pain | Colchicine | Improved knee pain and swelling, no abdominal pain with colchicine; concomitant occurrence hydatid disease |
Frigui et al. [24] | 35 M Origin: Tunisia HLA-B51 negative E148Q | Abdominal pain attacks with fever (15 years), hip and back pain (10 years), Recurrent oral ulcers (2 years), genital ulcers (1 year), thrombosis left popliteal vein (6 months) | Colchicine 1 mg/day and sulfasalazine 2 g/day | Diagnosed ankylosing spondylitis Decreased frequency of attacks at 5 year follow-up |
Bilginer et al. [25] | 17F Origin: Not stated M694V | Recurrent oral ulcers, erythema nodosum, fevers, arthritis (11 years) Secondary amyloidosis (acute) | Colchicine, nonsteroidals, methotrexate, occasional corticosteroids × 11 years Anakinra 1 mg/kg/day, colchicine, isoniazid prophylaxis | Minimal symptom improvement on initial treatment × 11 years 6 month and 1 year follow-up of anakinra, colchicine, isoniazid prophylaxis with resolution of clinical symptoms and acute phase reactants. Worsening proteinuria at 18 months |
Matsuda et al. [26] | 25 M Origin: Japan HLA-B51 negative E148Q/M694V | Buccal aphthous, genital ulcers, iridovirus, fever and thoracoabdominal pain due to pleural peritonitis (10 years) | Oral prednisolone, intermittently in past attacks Colchicine 0.5 mg/day | No improvement on prednisolone Symptoms improved on colchicine |
Birlik et al. [18] | 37 M, Origin: Turkey HLAB5 negative | Recurrent abdominal pain/fever (17 years), Recurrent oral ulcers (2 years), recurrent genital ulcers (1 year), erythema nodosum (9 months), sacroiliitis (acute) | Colchicine 1.5 mg/day and Sulfasalazine 2 g/day | Decreased acute phase reactants at 1 week; decreased acute attacks at 1 year |