Thrombotic microangiopathy in adult Still's disease.

Adult Still's disease (ASD) is a rare systemic disorder characterized by fever, arthralgia, cutaneous rash, and lymphadenopathy, with high polymorphonuclear leucocytosis and low glycosylated ferritinaemia. Kidney involvement has been reported rarely. We present a patient with ASD who developed haemolytic uraemic syndrome (HUS). The 42-year-old patient was admitted for unexplained fever related to ASD according to Yamaguchi's classification criteria. As Still's disease was resistant to prednisone, high-dose intravenous immunoglobulins (IV Ig) were administered. During the follow-up the patient developed acute renal failure and non-immune haemolytic anaemia with high levels of antiphospholipid antibodies (IgG anticardiolipin antibodies and anti-beta2 glycoprotein 1 antibodies). Renal biopsy disclosed thrombotic microangiopathy (TMA) with arteriolar and glomerular involvement. Treatment with steroids and intravenous IV Ig was reinitiated but renal function worsened towards end-stage renal failure. In this case, we suggest that antiphospholipid antibodies could have promoted arteriolar and glomerular TMA. HUS may be the cause of acute renal failure in Still's disease.

[Familial small-vessel vasculitis of the kidney]. / Vascularite familiale des vaisseaux de petit calibre des reins.

INTRODUCTION: Familial forms of small-vessel vasculitis has been reported in 14 families (including this one). CASES: A father and son were both diagnosed with renal vasculitis (pauci-immune crescentic glomerulonephritis). Both had antimyeloperoxidase autoantibodies, and there was no evidence of a common environmental factor. DISCUSSION: These cases suggest the role of constitutional factors in the pathogenesis of antineutrophil cytoplasmic antibody-associated vasculitis.

[Hemorrhagic fever with renal syndrome]. / Fièvre hémorragique avec syndrome rénal.

Puumala hantavirus is the most common hantavirus infection in Western Europe. The causative agent, Puumala virus, is a member of the Hantavirus genus in the Bunyaviridae family. The natural hosts of hantaviruses are chronically, but asymptomatic infected rodents, which transmit the virus to human in their excretions. Puumala virus is carried by the bank vole, clethrionomys glareolus. Hemorrhagic fever with renal syndrome (HFRS) caused by Puumala virus in France or Belgium is very similar to the previously described Nephropathia epidemica in Scandinavia. In most severe cases, the disease is clinically characterized by high fever of abrupt onset, headache, loin or abdominal pains, nausea and vomiting, and occasionally acute and transient myopia. Renal involvement results in transient proteinuria and hematuria and acute renal failure. Except for interstitial hemorrhage in the outer medulla, the renal histopathologic findings are unspecific and include prominent changes in the interstitium with interstitial oedema and inflammatory infiltrates. Thrombocytopenia, mild elevation of liver enzymes, and leukocytosis are typical laboratory findings. Spontaneous complete recovery is the rule. Laboratory diagnosis is primarily based on serology such as indirect immunofluorescence or capture enzyme--linked immunosorbent assays which detect IgM antibodies and an increased level of IgG antibodies against Puumala virus. Viral antigen may be demonstrated in the cytoplasm of renal tubular epithelial cells.

[Bartter's syndromes]. / Les syndromes de Bartter.

Bartter syndromes are defined as a family of inherited recessive autosomal tubulopathies. They are characterized by hypochloremia, hypokalemia, metabolic alkalosis associated with potassium renal leakage and normal blood pressure despite increased plasma renin activity. Three forms of the disease are identified as followed: 1) Gitelman syndrome or hypocalciuria hypomagnesemia syndrome is a mild form often discovered in childhood or teenagers in reason of tetany. It is an homogeneous disorder related to mutations of the genes encoding the thiazide-sensitive Na-Cl cotransporter located in the distal convoluted tubule. 2) Antenatal Bartter syndrome with hypercalciuria and nephrocalcinosis or hyperprostaglandin E syndrome is a severe form, often revealed by hydramnios, prematurity and growth delay. It is related to mutations of two types of genes encoding for transporters of Henle's loop: the bumetanide-sensitive cotransporter Na-K-2Cl (NKCC2) [type I] or the inwardly-rectifying potassium channel (ROMK) [type II]. 3) the classical form or type III Bartter syndrome, often revealed by dehydration in the first year of life, is associated with hypomagnesemia in 20% of cases and normal or increased calciuria. This form is related to mutations of CLCNKB gene encoding for a chloride channel in Henle's loop. This classification, in part related to the demonstration of mutations in the genes encoding for tubular chloride or potassium channels, does not fit all cases, overlapping syndromes are frequent. Moreover some endocrinological (diabetes) and neurological (deafness) abnormalities are sometimes associated with Bartter syndromes. Both phenotypic and genetic approach must help to the diagnosis of these tubulopathies.

[Granulomatous interstitial nephritis with acute renal insufficiency, a potential complication of intravesicular bcg therapy]. / Néphrite interstitielle granulomateuse avec insuffisance rénale aiguë, une complication potentielle de la BCGthérapie intravésicale.

Intravesical Bacillus Calmette-Guerin has proved to be the most effective treatment of superficial bladder carcinoma. This therapy may induce a renal failure of variable severity, sometimes associated with multiple organ failure. Renal prognosis of this complication is not well defined. We report on a 68 year-old male patient who developed granulomatous hepatitis and acute tubulo-interstitial nephritis with mononuclear infiltrates containing numerous epithelioid cells, following the third course of intravesical BCG. Eleven cases of renal impairment due to intravesical BCG have been previously described, as part of a systemic disease in seven patients. The potential pathophysiological mechanisms of this rare complication are precised.