[Immunonegative necrotizing glomerulonephritis: an atypical case of lupus nephropathy]. / Glomerulonefritis necrotizante inmunonegativa: un caso de nefropatía lúpica atípico.

We report here a case of systemic lupus erythematosus with severe, active pauci-immune necrotizing and crescentic glomerulonephritis. This patient had been diagnosed of a lupus nephritis type III previously and treated with steroids and azathioprine. After a renal symptomless period of nine years, she developed heavy proteinuria and hypertension. A second kidney biopsy was then performed. The pathological study disclosed a pauci-immune necrotizing crescentic and segmental glomerulonephritis. The absence of subendothelial and mesangial deposits was confirmed by both immunofluorescent microscopy and electron microscopy. The simultaneous immunological study showed normal ANCA levels while complement, anti-dsDNA and ANA were altered. The patient was treated with steroids and cyclophosphamide eith good response. This case points out the possibility previously recognized by others of an association between lupus and an pauci-immune necrotizing glomerulonephritis.

Takayasu's arteritis as a cause of renovascular hypertension in a non-Asian population.

Takayasu's disease (TD) is a chronic inflammatory arteritis which affects the aorta and its main branches and occasionally the pulmonary artery. Its cause is not known. Clinical manifestations are due to the intensity and location of arterial inflammation in the acute phase, as well as chronic arterial stenosis over time; 50% of patients have hypertension. Although TD appears to be more common in Asia, increasing numbers of patients of different races are observed in Western countries. The most important pathogenetic mechanism of hypertension seems to be through renal artery stenosis. We show here seven Caucasian hypertensive patients with TD and renovascular stenosis and arterial hypertension. One case was diagnosed in the acute phase of the disease, while in the others diagnosis was established in an advanced occlusive phase. Basic diagnosis was established by angiographic study, with biopsy confirmation in two cases. All patients had at least three of the six criteria listed as diagnostic of TD (by the American College of Reumatology). All patients had the following criteria: age of disease onset before 40 years (symptoms or findings related to TD), vascular bruits in different areas and all patients also had aortic and renal arterial stenosis with some lesions of the main aortic branches. Six of them had claudication of the extremities. We describe their clinical, analytical and angiographic features and also the therapeutic approach. We discuss the aetiopathogenic mechanisms of hypertension in this disease and suggest that TD is not an unusual cause of vasculorenal hypertension.