Antiphospholipid syndrome and polyarteritis nodosa: a diagnostic and therapeutic challenge

Abstract

Antiphospholipid syndrome (APS) can be primary, when it is not associated with another connective tissue disease, or can be secondary, especially associated with systemic lupus erythematosus. In a cohort of 1,000 APS patients, only seven (0.7 %) were associated with systemic vasculitis [1]. In the literature, there are few articles about the association of polyarteritis nodosa with APS comprising a total of 19 cases [2–8]. This study reports two additional cases with this rare condition, APS and polyarteritis nodosa. A 40-year-old Caucasian man was admitted after two epileptic crises. One year before, he reported unintentional weight loss of 47 kg and onset of livedo reticularis. His past medical history included arterial hypertension. Creatinine was 1.3 mg/dl. An arteriography showed multiple small aneurysms in the renal arteries. Antineutrophil cytoplasmic antibody (ANCA) and hepatitis B surface antigen were negative, and hepatitis B core antibody and hepatitis B surface antibody were positive. A diagnosis of polyarteritis nodosa was performed, and he was treated with prednisone 60 mg/day and azathioprine (150 mg/ day). After 5 years, he experienced abrupt ataxia, and magnetic resonance imaging of the brain revealed caudate nucleus and cerebellum infarction. At this occasion, he had positive IgG anticardiolipin ([120GPL) and IgM ([120MPL) antibodies, and aspirin (300 mg/day) was then added to the therapeutical scheme. He experienced complete recovery of this neurological symptom after one month. After 12 weeks, as IgG and IgM anticardiolipin antibodies continued highly positive, APS diagnosis was done. Currently, he is well, asymptomatic, using azathioprine (100 mg/day) and aspirin. The other case is a 43-year-old Caucasian man presented with ulcers on his legs, systemic hypertension and livedo reticularis. Since three years before, he had limb paresthesia in left leg. Electroneuromyography showed mononeuritis multiplex with sensitive and motor nerve fibers involvement in left ulnar nerve, and left posterior tibial and peroneal nerve. The histopathologic features of skin biopsy showed leukocytoclastic vasculitis with perivascular neutrophilic inflammation and necrosis of vessel wall. ANCA was positive. He had a good evolution, with healing of his ulcers. One year later, the patient presented infectious ulcer on hallux, which led to necrosis and amputation. He was treated with antibiotics, prednisone 70 mg/day associated with cyclophosphamide, but these treatments did not demonstrate any efficacy. A new ulcer biopsy was performed and demonstrated leukocytoclastic vasculitis with perivascular neutrophilic inflammation, necrosis of vessel wall and vascular thrombosis. Lupus anticoagulant was positive. APS diagnosis was then performed, and heparin followed by warfarin was started, and he experienced marked improvement of his ulcers. After 12 weeks, lupus anticoagulant continued positive. Nowadays, he is asymptomatic, without ulcers, using azathioprine 75 mg/day and warfarin with INR target of 3–4. C. A. M. Caldas Internal Medicine Department, Centro Universitario do Estado do Para, Universidade Federal do Para, Belem, PA, Brazil