Rapidly progressive glomerulonephritis with D-penicillamine

We present 3 cases of anti-myeloperoxidase, anti-neutrophil cytoplasmic antibody (MPO-ANCA)-positive rapidly progressive glomerulonephritis developed during the treatment with D-penicillamine (D-PC) for rheumatoid arthritis. Rheumatoid arthritis was diagnosed in these patients, and D-PC was administered to them at doses of 100, 200, and 300 mg per day for 32, 42, and 39 months, respectively. They developed proteinuria, hematuria, renal insufficiency, and anemia, and D-PC was stopped. On admission, MPO-ANCA was strongly positive in their sera. Renal biopsy showed glomerulonephritis with cellular crescents. Immunofluorescence examination revealed deposits of granular IgG, IgM, IgA, C1q, and C3 in the mesangium. The 3 patients were treated with steroid pulse therapy along with administration of anticoagulants, and cyclophosphamide was also used in 2 patients. Their renal function improved gradually and MPO-ANCA disappeared after immunosuppressive treatment.     

Rapidly progressive glomerulonephritis accompanying Legionnaires' disease

A rapidly progressive, crescentic glomerulonephritis with acute oliguric renal failure occurred simultaneously with legionnaires' disease (LD) in a 52-year-old man. The diagnosis of LD was based on a sixfold rise in indirect fluorescent antibody titer against Legionella pneumophila serogroup 4. Treatment with erythromycin lactobionate resulted in a clinical resolution of pulmonary manifestations. The impairment of kidney function, however, was progressive and within two weeks led to end-stage renal failure requiring regular hemodialysis. This observation suggests that LD may trigger severe acute glomerulonephritis.     

类狼疮性肾炎表现的感染性心内膜炎1例

患者男性, 47岁。以发热起病, 继而出现水肿、皮疹、蛋白尿、镜下血尿, 血肌酐1周内自264 μmol/L升至570 μmol/L, 补体C3 0.342 g/L, 补体C4 0.048 g/L, 抗核抗体1∶100。于当地医院行肾组织活检, 免疫荧光提示多种免疫复合物在肾脏沉积, 光镜下呈现新月体型肾炎。考虑系统性红斑狼疮, 狼疮性肾炎。予静脉输注甲泼尼龙500 mg 治疗3 d, 序贯口服泼尼松60 mg 每日1次, 患者血肌酐下降至416 μmol/L后再次升高(517 μmol/L), 故转诊至我院。入院体检发现主动脉瓣听诊区舒张期吹风样Ⅲ～Ⅳ级杂音, 遂行超声心动检查, 见主动脉瓣赘生物形成, 考虑感染性心内膜炎相关肾小球肾炎。予青霉素肌肉注射(4 MU, 每12小时1次)10 d序贯头孢曲松静脉输注(2 g, 每日1次)6周, 糖皮质激素逐渐减量至30 mg 每日1次, 行主动脉瓣置换术, 糖皮质激素每2周减量5 mg 每日1次。术后患者血尿、蛋白尿消失, 血肌酐下降至98 μmol/L。     

急进性肾炎综合征伴颅内出血

42岁男性,病程2个月,临床表现消瘦、贫血伴急进性肾炎综合征,p-ANCA及MPO-ANCA高滴度阳性,既往无高血压病及凝血功能异常。住院期间突发口角歪斜、左侧肢体偏瘫,急诊头颅CT提示右侧基底节区出血,脑组织造影未见脑血管畸形,诊断微型多动脉炎并发颅内出血,经止血、脱水及甲泼尼龙等控制原发病治疗后患者症状逐渐缓解。     

Rapidly progressive glomerulonephritis and monoclonal gammopathy

A case of rapidly progressive glomerulonephritis associated with nephrotic syndrome, hematuria, and edema is reported. Monoclonal IgG-lambda was found in the serum and urine. Renal biopsy revealed diffuse proliferative glomerulonephritis with crescent formation. Immunofluorescent study revealed IgG and lambda in a focal segmental distribution. Subepithelial humps were found on electron microscopic examination. A spectacular feature of the deposits was the presence of organized linear fibrils within the humps. Similar fibrils were found in the mesangium and urinary space. Renal function deteriorated rapidly, necessitating hemodialysis in eight months. In addition to the present case, 24 cases of glomerulonephritis associated with "benign" monoclonal gammopathy reported since 1970 are reviewed, and the potential causal relationship between monoclonal gammopathy and glomerular involvement is stressed.     

Sarcoidosis presenting as antinuclear antibody positive glomerulonephritis.

A 43 year old woman who initially presented with the nephrotic syndrome, glomerulonephritis, and antinuclear antibodies (ANAs) was given the diagnosis of systemic lupus erythematosus (SLE). One year later the patient developed progressive subcutaneous nodules on her forearms, with histopathology of non-caseating granulomas. Further evaluation of the patient showed mediastinal lymphadenopathy and interstitial lung disease with numerous granulomas, establishing the diagnosis of sarcoidosis. The presence of autoimmune antibodies and glomerulonephritis has been reported in sarcoidosis, but this case is believed to be the first in which both glomerulonephritis and ANAs are present in a sarcoid patient.     

IgA nephropathy with crescentic glomerulonephritis and ANCA positive

We present a case of IgA nephropathy diagnosed by renal biopsy that presents after 2 years of folow-up an impairment of the renal function associate histoligically to a crescentic glomerulonephritis. The immunologic determinations showed of high titers of antineutrophil cytoplamic antibodies (ANCA) (P-ANCA IgG antiMPO and P-ANCA IgA anti-MPO). The patient began treatment with haemodyalisis and one year later she received a cadaveric kidney transplantation with good result. Two years later she had normal urine sediment, negative proteinuria, normal renal function but high titers of IgG and IgA PANCA anti-MPO. In summary, we believe that the determination ANCA in acute renal failure due to IgA nephropathy can indicate the existence of a IgA crescentic glomerulonephritis superimposed or an associated small vessel vasculitis and it confers a worse prognosis. The positive maintenance of IgG P-ANCA anti-MPO IgG anti-MPO titers during the course suggests that the sequential determination of ANCA in this entity is not useful to the monitoring of the clinical activity.     

Rapidly progressive glomerulonephritis in a 68-year-old man

A 68-year-old male with macroscopic hematuria and constitutional symptoms as fever and weight loss. There was nothing interesting in the anamnesis or in the physical exploration. The laboratory test had an elevation of creatinine of 4 mg/dL and ten days before it had been 1.4 mg/dL. In the urine analysis: proteinuria of 1.5 G/24 h, and hematuria. On the second day we made a renal biopsy where we could seen segmental glomerular necrosis and crescent fromation in 80% of the glomeruli. In the immune study c-ANCA anti-PR3 was positive. In the complementary studies we didn't find other organs affected. With the diagnosis of pauci-immune glomerulonephritis limited to the kidney we began treatment with corticosteroids and cyclophosphamide. As the renal function was severely affected the patient needed one dialysis session. We began with 1 g intravenous methylprednisolone daily for 3 days followed by oral prednisone 60 mg daily tapering to 10 mg daily by 3 months. This was combined with 150 mg oral cyclophosphamide daily. Seven plasma exchanges were performed. At the beginning of treatment creatinine was 7 mg/dL, it was decreasing rapidly and three week after cretinine was 3 mg/Dl and he was asymptomatic. One year after treatment, creatinine is 1.4 mg/dL and the urine analysis is normal, C-ANCA are negative.     

Rapidly progressive glomerulonephritis. A clinical and pathologic study.

Twenty-nine cases of rapidly progressive glomerulonephritis were reviewed. In all cases there was less than three months between the onset of renal symptoms and renal biopsy. The serum creatinine was greater than 2.5 mg/100 ml at the time of biopsy, and the histology showed a 50 per cent or greater incidence of crescents in the glomeruli. Infectious or febrile episodes were present in 21 cases, microscopic hematuria was noted in 15 and proteinuria exceeding 2.5 g/24 hours in eight. Oliguria less than 500 ml/24 hours was present in 20 cases and dialysis was required in 22. In 10 cases there was sustained improvement; in the remainder the disease progressed or the patient died. The prognosis was related to the number and size of the glomerular crescents. Histologically cases fell into two main groups, one with predominantly extracapillary proliferation and the other with endo- and extracapillary proliferation. In the first group the disease was histologically and clinically more severe, and Immunofluorescence histology was heterogeneous and often non-specific except for three cases in which there were linear immunoglobulin deposits. In the second group the lesions were less severe and immunoglobulin deposits were common. Electron microscopy in 14 cases was confirmatory, and also demonstrated capillary rupture and necrosis of podocytes in some loops. Transition from group I to group II was observed in serial biopsy specimens in one case. The histologic and immunofluorescent heterogeneity suggest that rapidly progressive glomerulonephritis is the end result of several different pathogenetic mechanisms.     

Rapidly progressive glomerulonephritis with glomerular crescent formation in rheumatoid arthritis

Summary Systemic vasculitis is a well-known complication of rheumatoid arthritis (RA). Rapidly progressive glomerulonephritis is commonly associated with vasculitis syndromes but rarely found in RA. This report describes two RA patients with complicating systemic vasculitis who developed rapidly progressive glomerulonephritis with glomerular crescent formation. One recovered after treatment with corticosteroids and cytostatic drugs and the other died of severe systemic vasculitis despite similar therapy.     

Rapidly progressive glomerulonephritis:classification, pathogenesis and clinical management

Rapidly progressive glomerulonephritides (RPGN) belong to a heterogeneous group of inflammatory kidney diseases which are commonly associated with systemic vasculitic syndromes. Renal histology is characterized by necrotizing lesions within the glomerual tuft and extracapillary proliferation, in most cases leading rapidly to renal failure. The etiology and pathogenesis are only partly elucidated. Since irreversible renal scaring develops within days to weeks, RPGN represent a nephrological emergency necessitating urgent diagnostic evaluation and rapid institution of effective therapy. New onset nephritic sediment combined with concomitantly deteriorating excretory renal function should lead to immediate nephrological consultation. Autoimmune serology and particularly renal biopsy are of the utmost importance for rapid diagnosis. Most forms of RPGN are treated with immunosuppressive regimens which generally consist of high dose steroids in combination with the alkylating agent cyclophosphamide. Some forms also require the use of plasma exchange therapy. Rapid diagnosis and early therapy improves both renal and overall outcome in the affected patients.     

Rapidly progressive lupus glomerulonephritis and concomitant microangiopathy in an adolescent

We describe our experience managing a 16-year-old girl with systemic lupus erythematosus (SLE) who presented concomitantly with rapidly progressive glomerulonephritis (RPGN) and a thrombotic microangiopathic hemolytic anemia (TMAHA). Her renal biopsy showed evidence of diffuse proliferative glomerulonephritis without glomerular microthrombi. The patient was treated with a combination of intravenous corticosteroids and cyclophosphamide, as well as plasmapheresis, with an excellent response resulting in complete disease remission. The purpose of our report is to make health professionals more aware of TMAHA as a complication of SLE, since the occurrence of TMAHA may confuse the clinical picture, and since its treatment with plasmapheresis is life saving, if performed early.     

Giant cell carcinoma causing rapidly progressive respiratory failure as the presenting feature of AIDS

The incidence of lung cancer has been increasing among HIV-positive patients. The majority of these cases were in patients previously diagnosed as HIV-positive and treated with highly active antiretroviral therapy (HAART). Here, we report a 56-year-old male patient with lung cancer, who was diagnosed as HIV-positive after the onset of neck pain and lumbago and thus, was not treated with anti-AIDS therapy. The patient developed rapidly progressive and fatal respiratory failure. Autopsy demonstrated giant cell carcinoma of the lung responsible for carcinomatous lymphangitis. This case highlighted the possibility that pulmonary carcinogenesis in HIV-positive patients is not necessarily associated with HAART therapy.