[Antiphospholipid antibody syndrome as the cause of clinical rapidly progressing vasculopathy]

For 3 weeks a 54-year-old man had been unsuccessfully treated with antibiotics for ulcerating tonsillitis with fever, weight loss, occasional headaches, and hypertension. A year before he had suffered a transitory cerebral ischaemic attack. On admission he had a fever of 38.6 degrees C, blood pressure of 185/100 mmHg and a nonspecific 2/6 apical systolic murmur.

Erythrocyte sedimentation rate was 100/120 mm, C-reactive protein 1.5 mg/dl, serum creatinine 2.45 mg/dl, urinary protein 0.8 g/d. Tests for antinuclear antibodies (ANCA) against neutrophil granulocytes and for native DNA antibodies were negative, as were serological tests for Coxsackie virus, Cytomegalovirus and Epstein-Barr virus. Sonography and computed tomography revealed thrombosis along the entire aortic wall, renal artery stenosis and a contracted left kidney. Transoesophageal echocardiography demonstrated small thrombi on the aortic valve leaflets. Coloscopy showed ulcerative colitis.

The patient was anticoagulated. Local cold produced livedo reticularis. Tests for cardiolipin antibodies in serum and a skin biopsy provided the diagnosis of antiphospholipid antibody syndrome (APS). As the skin biopsy showed vasculitis, immunosuppressive treatment was started. This stabilized the condition and he was discharged to be followed by his general practitioner.

APS can cause complex symptoms in many organs, but effective treatment (anticoagulation, corticosteroids, immunosuppressives) is available, as this case demonstrates.