Systemic sclerosis complicated by procainamide-induced lupus and antiphospholipid syndrome.

P is one of the major causes of drugchest pain and was admitted to our hospital. An induced lupus erythematosus (DIL) [1]. Patients taking electrocardiogram showed monomorphic ventricular procainamide for prolonged periods often develop tachycardia (VT; heart rate 150/min), which was sucanticardiolipin antibodies, as well as anti-DNA and cessfully treated with 600 mg of i.v. procainamide. anti-histone antibodies [1–3]. Some of these patients Echocardiogram and right ventriculogram with cardiac manifest thrombosis, as a part of drug-induced anticatheterization showed dilatation of the right atrium phospholipid syndrome (DI-APS) [2–6 ]. However, the and right ventricle. A biopsy from the right ventricular frequency of thrombotic events in these patients is myocardium revealed localized myocardial fibrosis. considered to be less than that seen in antiphospholipid Treatment with 2000–3000 mg/day of procainamide syndrome (APS) associated with systemic lupus erythsuccessfully suppressed ventricular arrhythmias. ematosus (SLE) [3–5, 7]. In May 1993, he noticed a new skin ulcer on his left Systemic sclerosis (SSc) is another autoimmune distoe. Beraprost sodium (120 mg/day) and i.v. lipoease of unknown aetiology. Its primary site of injury prostaglandin 1 ( 1 ; 10 mg/week) did not is the vascular system, especially at the microvascular improve the ulcer, and he was admitted to our hospital level. Raynaud’s phenomenon and digital tip ischaemia on 16 July 1993. Physical examination demonstrated are commonly seen [8]. However, digital gangrene skin sclerosis of the face, extremities and trunk. An which often requires amputation ensues in a few SSc ulcer with necrotic tissue on his left second toe was patients [8], mainly in patients with limited cutaneous found (Fig. 1). Pulsation of dorsal pedal arteries was involvement [9]. not palpable bilaterally. Tenderness was observed in We present a patient with SSc with diffuse cutaneous his proximal interphalangeal and metacarpophalangeal involvement (dcSSc) who later developed a toe ganjoints. Facial erythema, alopecia and oral ulcers were grene, arthritis and pancytopenia as the manifestations not found. Laboratory testing revealed pancytopenia of APS and lupus-like syndrome after the administra(white blood cell count 3600/ml, haemoglobin 8.6 g/dl tion of procainamide for the treatment of a ventricular and platelet count 80 000/ml ), a prolonged activated arrhythmia. partial thromboplastin time (aPTT >150 s) and pro-