[Symptomatic treatment of nephrotic syndrome]. / Traitement symptomatique du syndrome néphrotique.

OBJECTIVES OF SYMPTOMATIC TREATMENT: The goal is to maintain quality of life, prevent immediate complications (thromboembolic events, infection, drug reactions), prevent late complications related to atherosclerosis, and limit the progression of the chronic renal failure. THERAPEUTIC ARMAMENTARIUM: Six categories can be described. i) A reduction in proteinuria, essential for controlling the intensity of other manifestations, can be improved with a normal protein content (1 g/kg ideal weight/d) low-salt diet, strict blood pressure control, and most importantly, CEI given alone or in combination with AA2. ii) Restoration of a normal extracellular fluid (edema and high BP) can be achieved by low sodium intake and loop diuretics in fractionated increasing doses (sometimes with combination regimens). It is advisable to keep blood pressure below 125/75 mmHg. iii) Prevention of thromboembolic events (risk level dependent on urine protein output) relies on antivitamin K anticoagulants and low-molecular weight heparins. iv) Adapted prescription of protein-bound drugs. v) Lowering LDL-cholesterol, a risk factor for atherosclerosis, with an adapted diet and HMG CoA inhibitors. vi) Prevention of chronic renal failure. The development and course of chronic renal failure depend not only on the histological glomerular lesion and/or the etiology but also on supplementary glomerular and tubulointerstitial damage directly related to the degree of proteinuria. MORE THAN SYMPTOM RELIEF: Symptomatic treatment of nephrotic syndrome must be considered as an integral part of a rigorous goal-oriented therapeutic strategy.

Massive proteinuria as a main manifestation of primary antiphospholipid syndrome.

Renal involvement in antiphospholipid syndrome (APS) is increasingly reported. So far, massive proteinuria as the principal feature of primary APS (PAPS) has not been well documented. We describe 3 patients with PAPS and massive proteinuria. Renal biopsy was performed in all 3, and features consistent with membranous and focal segmental glomerulopathy were disclosed. These histological lesions were not yet reported in PAPS. We conclude that the spectrum of renal lesions in PAPS is diverse and that it should be considered in the differential diagnosis of patients with massive proteinuria.

Membranous nephropathy in primary antiphospholipid syndrome: description of a case and induction of renal injury in SCID mice.

Primary antiphospholipid syndrome (PAPS) is a recently recognized clinical entity encompassing the combination of thromboembolic phenomena, thrombocytopenia and recurrent abortions in the presence of antiphospholipid antibodies. We present a patient with PAPS accompanied by renal involvement, manifested as membranous nephropathy, as proven by a renal biopsy. To investigate further the possible association between PAPS and the renal lesions we attempted to induce similar renal manifestations by transferring peripheral blood lymphocytes (PBL) from this patient to severe combined immunodeficiency (SCID) mice. The mice transfused with PBL from the affected patient exemplified antiphospholipid antibodies (aPL) following which a renal lesion consistent with the human membranous nephropathy lesion was precipitated. This study substantiates the role of aPL as possible inducers of renal damage.

Captopril induces correction of postrenal transplant erythremia.

Kidney transplant patients may develop post-transplant erythremia (PTE), and in order to avoid thromboembolism venesection, anticoagulation and native kidney removal have been suggested. We propose captopril as an alternative therapy for PTE. Seven hypertensive PTE patients, aged 42 +/- 10 years with stable renal function, were investigated to exclude primary or secondary polycythemia. All patients manifested true erythrocytosis [red blood cells (RBC) mass greater than 20% of predicted level] with concomitant increases in hematocrit and hemoglobin levels. Captopril was introduced in gradually increasing doses up to 75 mg/day under careful monitoring of blood pressure and renal function. Weekly follow-up was arranged to evaluate drug efficacy. After captopril, a significant reduction with normalization of the RBC mass (42 +/- 4 vs 31 +/- 5 ml/kg: P less than 0.005) was observed. The RBC counts and hematocrit and hemoglobin levels also decreased. One patient had recurrent erythrocytosis after captopril withdrawal. Captopril may be a simple, effective, and non aggressive treatment for postrenal transplant erythremia.

[Dilatation of bile ducts in polycystic kidney disease in adults]. / Dilatation des voies biliaires au cours de la polykystose rénale de l'adulte.

We report the cases of 4 adult patients with polycystic kidney disease and dilatation of bile ducts. Dilatation involved the extra-hepatic bile ducts in all 4 cases and also affected the intra-hepatic bile ducts in 3 cases. A prospective ultrasonographic study in search of biliary tract abnormalities was undertaken in 40 patients with dominant polycystic kidney disease. No bile duct dilatation was found in this series, which indicates that the lesion is rare. The 4 cases reported here increase the collection of hepatobiliary lesions associated with polycystic kidney disease of adults.

[Fatal acute pneumonopathies in disseminated lupus erythematosus]. / Pneumopathies aiguës mortelles au cours du lupus érythémateux disséminé.

Two cases of systemic lupus erythematosus (SLE) complicated by pneumonia which resulted in death are reported. The first patient, a 21-year old woman, died of acute diffuse lupus pneumonia; the initial and unusual radiological image of "multiple balloons" progressed within 2 months to terminal interstitial fibrosis. The second patient, a 60-year old woman, died of infection on an interstitial pneumonia which turned into severe fibrosis within 16 months. Acute or chronic lupus pneumonia is uncommon, but it may follow a very serious course. Clinically, true lupus pneumonia must be distinguished from all other types of lung involvement in SLE, such as infection, pulmonary haemorrhage or oedema, iatrogenic pathology, thromboembolic disease, etc. The pathogenetic mechanism of pulmonary lesions directly related to SLE is obscure, although some lung biopsy specimens have shown positive immunofluorescence. Concerning treatment, the initial response to corticosteroid therapy is usually very good, especially in the acute forms of the disease. However, in severe cases immunosuppressive drugs or even plasma exchanges must be added to steroids. For treatment to be rapidly initiated the diagnostic procedures must be completed in the early stages of the disease, involving, when necessary, surgical lung biopsy.

[Sicca syndrome with severe renal insufficiency]. / Syndrome sec avec insuffisance rénale sévère.

The interstitial nephritis associated with sicca syndrome is usually symptomless or responsible for mild renal impairment. The authors report five cases in which renal failure was severe, requiring haemodialysis in two patients. The physiopathological mechanisms and the treatment of interstitial nephritis in sicca syndrome are discussed. Corticosteroids improved renal function in three of these five patients.

[Pure hypertensive nephropathy associated with malignant arterial hypertension in disseminated lupus erythematosus. Case with a favorable outcome]. / Néphropathie hypertensive pure, contemporaine d'une hypertension artérielle maligne au cours d'un lupus érythémateux disséminé. Une observation d'évolution favorable.

The authors report the association of a clinically florid form of lupus with malignant hypertension and renal failure. The renal histology revealed lesions of nephroangiosclerosis, with virtually no signs of lupus proliferative glomerulonephritis. With a follow-up period of 42 months, the renal function has returned to normal, although steroid treatment has been interrupted for 33 months. However, intensive antihypertensive treatment is still required. This unusual case is discussed in the light of previous reports in the literature of the association of disseminated lupus erythematosus and malignant hypertension, which is nevertheless a rare entity.

[Current status of Wegener's syndrome. Apropos of 2 cases]. / Actualité du syndrome de Wegener. A propos de 2 observations.

In the context of two recent cases, the authors briefly review the clinical, laboratory and anatomical features of Wegener's granulomatosis and, in particular, its clinical course in response to treatment. These 2 cases include fairly original features: one case presented with abundant haemoptysis with a radiological picture of extensive diffuse bilateral pneumonia; the other case presented with a very large pleural effusion with no E.N.T. involvement whatsoever. The authors then summarise the classical features of this severe form of granulomatous vasculitis, which essentially affects the lungs, the kidneys and the ear, nose and throat. The lesions have a typical histological appearance. The major interest of this disease lies in its treatment. The natural evolution of this disease is very serious with a mean survival of 5 months. However, with immunosuppressant and corticosteroid treatment, a complete remission is obtained and maintained in more than 90% of cases. The therapeutic protocol used, as in one of the present cases, prevents the development of the lesion responsible for the very serious prognosis, irreversible renal failure.

[Mytomycin C nephrotoxicity. 3 new cases and review of the literature]. / La néphrotoxicité de la mitomycine C. Trois nouvelles observations et revue de la littérature.

Three women with adenocarcinoma of cardia, adenocarcinoma of liver or ileum carcinoid tumor, respectively, were treated with mitomycin C (MMC) and 5-fluoro-uracil (5-FU). Two patients had renal impairment 6 and 11 months, the third as early as 8 weeks after initiation of MMC therapy. In the three cases, blood transfusions appeared to play an aggravating role inducing respiratory distress, microangiopathic hemolytic anemia and thrombocytopenia, and to accelerate progression of renal failure. Death occurred in the first patient; terminal renal insufficiency necessitated hemodialysis treatment in the second, and after 10 plasma exchanges, renal failure remained stable in the third. Light and electron microscopy study of the kidney revealed renal lesions compatible with thrombotic microangiopathy in all cases. Mesangiolysis with swollen nuclei of endothelial cells in most glomeruli was also seen in two cases. We compared our observations with the findings found in 30 previously reported cases. These observations substantiate the renal toxicity of mitomycin C.