Malignant Hypertension and Antiphospholipid Syndrome

Dr. A.E. Sirvent, Avda Orihuela 4, 6 D, E-03006 Alicante (Spain) Dear Sir, The kidney is now recognized as another target organ in the antiphospholipid syndrome (AS) [1]. Renal involvement is consistent with thrombosis of the major renal vessels and noninflammatory microvascular disease [2-4]. In the clinical findings renal failure has been emphasized over systemic hypertension. We report here on a patient with systemic malignant hypertension and throm-botic microangiopathy (TM) with no other occlusive complications. A 29-year-old man was hospitalized with acute renal failure during a malignant hypertensive episode, after developing hypertension in the 3 months prior to admission. Thrombocytopenia was also detected. Physical examination showed: blood pressure 230/ 130 mm Hg, grade IV retinal changes, no abdominal bruits were heard; the signs were compatible with congestive heart failure. The most significant laboratory findings were: hemoglobin lOg/dl, WBC 14,000/μl (normal differential count), platelets 47,000/μl, normal coagulation tests, fíbrinogen 490 mg/dl and schistocytes on a peripheral blood smear. Urea was 35 mmol/l, creatinine 884 μmol/l, LDH 971 U/l, haptoglobin 11 mg/dl, and urine contained proteinuria 2.1 g/day, 10-15 erythrocytes/hpf. Chest X-ray revealed pulmonary edema and cardiomegaly, and echo-cardiography indicated left ventricular hypertrophy, normal ejection fraction and mild mitral regurgitation. The abdominal ultrasound showed a right kidney of 11.9 cm and a left kidney of 8.7 cm. No evidence of kidney infarction was seen in the renal CT scan. The magnetic resonance angiogram disclosed normal aorta and renal arteries. The following laboratory data were negative or normal: urine cultures, urine catecholamines and VMA, HBsAg, serology for HCV, cryoglobulins, rheumatoid factor, ANCA, anti-GBM, ANA, anti-DNA, anti-SSA (Ro), anti-SSB (La), anti-Sm, anti-RNP antibodies, C3, C4, VDRL, brain CT, cystography, lupus anticoagulant; anticardiolipin antibodies (aCL) IgG 63.3 GPL/ml (n.v. < 23), IgM 3.1 MPL/ml (n.v. < 11), and repeated after 8 weeks, IgG 40 GPL/ml (ELISA). Antiplatelet antibodies were positive. The kidney biopsy showed TM; no deposits were noted in the immunofluores-cence study. Blood pressure was controlled. An increase in platelets and normalization of LDH was achieved following plasma exchange with FFP as the replacement fluid; upon withdrawal of the plasma exchange, thrombocytopenia recurred. After receiving the aCL determination