Rheumatoid arthritis with ulnar deviation of the metacarpal-phalangeal joints, button hole deformity, and swan neck deformity. 

Rheumatoid arthritis with ulnar deviation of the metacarpal-phalangeal joints, button hole deformity, and swan neck deformity. 

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Patient: Male, 66 Final Diagnosis: Acquired hemophilia A Symptoms: Polyarticular flare Medication: — Clinical Procedure: — Specialty: Rheumatology Objective Challenging differential diagnosis Background Acquired hemophilia A (AH) is a rare hemorrhagic diathesis, characterized by the presence of autoantibodies directed against the pro-coagulant ac...

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... patient was a 66-year-old man who was a 30-pack-year long-time tobacco-smoker who quit smoking 10 years ago, and with a 25-year history of Leo Buerger disease, currently in remission. He had been followed up for 20 years for deform- ing ( Figure 1) and severe RA (Figure 2), with positive rheumat- ic serum (positive for rheumatoid factor and anti-cyclic citrul- linated peptide [Anti-CCP]). He had a destructive rheumatoid arthritis, with no systemic impairment, but with an impor- tant functional deterioration (difficulty eating, holding a glass, and walking). RA was in low-disease activity at 20 mg daily of leflunomide and 5 mg of prednisone per day. However, the patient presented a polyarticular flare involving the metacar- pal-phalangeal (MCP) and the proximal inter-phalangeal (PIP) joints, the left elbow and the right knee were warm and swol- len on clinical examination, and with spontaneous ecchymotic patches. There were no other extra-articular signs. The general condition was maintained and there were no symptoms of an infection. The articular puncture of this knee yielded a moder- ate amount of hematic fluid that did not coagulate ( Figure 3). Cytological analysis did not show any abnormalities except for a significant presence of red blood cells, which was also found abundantly in the other cell lines. There were no microorgan- isms or microcrystals. This hemarthrosis suggested a synovial local disease (e.g., villonodular synovitis or synovial angioma). However, in the presence of spontaneous bruising, a general disorder was suspected, especially an acquired abnormality of hemostasis including thrombocytopenia, thrombopathy, capil- lary fragility secondary to long-term corticosteroid use, a def- icit in factor II, V, VII, IX, and X in the context of hepatocellu- lar insufficiency caused by leflunomide, or hypo-avitaminosis K, and finally, acquired hemophilia through the presence of a circulating anticoagulant; while noting the absence of trauma or taking an anticoagulant. The imaging of the knee did not detect synovial anomalies. Platelet and leukocyte levels were normal in the blood count, which nevertheless revealed hypo- chromic and microcytic anemia at 10.6 g/dL of hemoglobin, originating from an iron deficiency. Activated partial throm- boplastin time (APTT) was lengthened to 49 s (normal range 25-35 s) and not corrected by the addition of control plasma. However, prothrombin time (Quick's test), fibrinogen level, vi- tamin K-dependent factors, and hepatic function tests were without abnormalities. In contrast, factor VIII was collapsed at 7% and the anti-factor VIII antibody was positive at 19 Bethesda Units (BU)/mL. The diagnosis of acquired hemophil- ia A with anti-factor VIII inhibitor was thus retained. However, the patient had never presented extensive mucosal cutaneous hemorrhages such us epistaxis, gingivorrhagia, or hematuria, nor was there a history of melena or another digestive bleed- ing. The anti-nuclear ac was negative and the pelvic thoraco- abdominal computerized tomography scan for other sites of bleeding or lymphoma was normal. Tumor markers sugges- tive of para-neoplastic origin were negative. With regard to RA, the C-reactive protein (CRP) was 26 mg/L and the eryth- rocyte sedimentation rate was 45 mm/1 st hour. The Disease Activity Score (DAS28 CRP ) was 6.32 and exhibited a very active RA. The patient was given 240 mg of methylprednisolone in bolus IV infusion for 3 days combined with a recombinant ac- tive factor VII infusion (initial dose of 90 μg/kg body weight every 3 h by an electrically operated syringe pump). The dose of 90 μg/kg was given every 12 h for 12 days until factor VIII was normalized). Rituximab was introduced according to the therapeutic modalities of RA at a dose of 1 g by giving 2 infu- sions at 2-week intervals to better control both the severely progressive RA and acquired hemophilia. Simultaneous with in eradicating the inhibitor, the patient was given methotrex- ate at 20 mg per week combined with 7.5 mg of prednisone, which could be gradually lowered to 4 mg/day after 6 months. A regular follow-up was recommended to assure optimal ther- apeutic compliance. The patient was seen at the consultation every 2 weeks for 3 months, then once every month. Our pa- tient was warned about the severity of his hematological pa- thology and that he had to immediately go to the hospital if he experienced any abnormal symptoms and he was given the phone numbers of the attending physicians. The evolution was favorable. After 6 months of treatment with rituximab, fac- tor VIII was 75% and the RA was in remission (DAS28 CRP =2.3). However, the reappearance of the anti-factor VIII inhibitor was 8-11 BU/mL, thus justifying a second cycle of ...

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