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 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
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PMID:[Immunonegative necrotizing glomerulonephritis: an atypical case of lupus nephropathy]. 1270 80

We report here two cases (72-year-old woman, 77-year-old-woman) with perinuclear anti-neutrophil antibody (P-ANCA) positive microscopic polyarteritis nodosa (mPN). Both patients presented with generalized convulsion, consciousness disturbance and severe hypertension several days after the administration of high dose methylpredonisolone followed by oral predonisolone. CT brain scan showed hypodensity area on bilateral posterior lobes. MRI T2 weighted image and FLAIR image showed increased signal intensity on the occipital gray and white matter. Although diffusion weighted MRI disclosed slightly high signal intensity, apparent diffusion coefficient (ADC) value at the occipital gray and white matter remained normal or even elevated. These findings, which were consistent with vasogenic edema, lead us to diagnose them as suffering from reversible posterior leukoencephalopathy syndrome (RPLS). After the second administration of high dose methylpredonisolone, their symptoms and signs, together with the MRI findings, gradually improved. To our knowledge, this is the first report concerning RPLS in a patient with mPN.
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PMID:[Reversible posterior leukoencephalopathy syndrome in two patients with microscopic polyarteritis nodosa]. 1273 85

Wegener's granulomatosis is a form of systemic vasculitis of unknown etiology that affects the small blood vessels. The disease is characterized by the formation of granulomas on small and medium blood vessels, and often affects the respiratory tract and renal system. In 86% of diagnosed cases, renal compromise is in the form of glomerulonephritis. Wegener's granulomatosis can occur at any age, principally during the 4th and 5th decades of life, and 85% of cases occur over the age of 19 years. In the United States about 3 per 100,000 cases are reported, with a major occurrence in males. The present report is about a case of a 12 year old female with Wegener's granulomatosis of 18 months of evolution, with symptoms such as epistaxis, generalized edema, cough, dyspnea, hemoptysis, high blood pressure and macroscopic hematuria. Out of the five criteria for the diagnosis of Wegener's granulomatosis, she had perinuclear anti-neutrophil cytoplasmic antibody positive to serum p-ANCA mark, which is the least frequent, which makes more difficult the diagnosis. Over a period of five years, in our hospital, we have diagnosed 11 patients with Wegener's granulomatosis, and the present case is the only pediatric case.
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PMID:[Wegener's granulomatosis. Report of a pediatric case and review of the literature]. 1282 92

We report an anti-GBM antibody-positive crescentic glomerulonephritis patient who benefitted from maintenance hemodialysis 4 months after the initial treatment, which included steroid pulse therapy and plasma exchange. A-29-year-old male was referred to our hospital because of high fever, abnormal urinary findings (leukocytes 3+, protein 2+, occult blood 3+) and a moderate degree of azotemia(S-Cr 2.9 mg/dl). C-reactive protein (CRP) was 18.9 mg/dl and antibiotics were administered intravenously for 7 days under the diagnosis of pyelonephritis. High fever persisted, however, and S-Cr increased to 9.2 mg/dl even though a sufficient volume of urine was maintained. Blood and urine cultures were negative for bacteria. A kidney biopsy was performed and cellular crescents were observed around the glomeruli. No abnormal finding was observed in the lung and the nasopharyngeal region. To treat the crescentic glomerulonephritis, steroid and cyclophosphamide were administered while hemodialysis was carried out simultaneously. Although P-ANCA and C-ANCA were negative, anti-GBM antibody was proven to be positive thereafter (169 U) and six sessions of plasmapheresis were additionally performed to remove the antibody. Two months after the last plasmapheresis, the reduced urine volume (300 ml/day) gradually returned to normal. Hemodialysis was terminated because the S-Cr concentration reached a plateau at 4 mg/dl. Repeated biopsy revealed marked glomerulosclerosis, hence hypertension treatment and a low protein diet were ordered. In conclusion, residual renal function might improve even after 4 months of hemodialysis in cases of intensively treated anti-GBM-positive crescentic glomerulonephritis, though consecutive renoprotective therapy is required.
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PMID:[A case of anti-GBM-antibody positive rapidly progressive glomerulonephritis who was weaned from hemodialysis after combination therapy with steroid and plasmapheresis]. 1640 34

Systemic sclerosis is an autoimmune disease characterized by fibrosis of the skin and internal organs. Raynaud's phenomenon generally precedes other disease manifestations. The distribution of skin lesions and the internal organ involvement are the basis for the classification into limited and diffuse forms of the disease. Clinically evident renal disease is observed in 10-40% of patients. The most common renal presentation is renal crisis, characterized by acute onset of renal failure and severe hypertension; some patients remain normotensive, showing microangiopathic hemolytic anemia. Renal complications due to penicillamine may occur in some patients. Finally, ANCA-associated glomerulonephritis is a rare complication of the disorder. In spite of treatment with ACE inhibitors, 20-50% of patients with renal crisis progress to end-stage renal disease. In the absence of a specific therapy, there is accumulating evidence supporting the effectiveness of prostacyclin derivatives, antifibrotic and immunosuppressive drugs. The evidence is strong that the ACE inhibitors that are used in renal crisis are disease modifying. In our series including 193 patients with systemic sclerosis, renal involvement was observed in 19 patients; 11 presented renal crisis (hypertensive in 8; normotensive in 3); 5 had chronic nephropathy; 2 developed penicillamine-induced nephrotic syndrome, and 1 ANCA-associated glomerulonephritis. Renal disease occurs in a minority of patients with systemic sclerosis, and may have a variable clinicopathological picture. As renal involvement is associated with a worse prognosis, careful monitoring of blood pressure, urine chemistry and renal function is required, particularly in patients with diffuse skin disease.
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PMID:[Renal involvement in systemic sclerosis]. 1765 1

The scleroderma renal crisis is characterized by acute onset of severe hypertension and by rapidly progressive hyperreninemic renal failure. There is, however, a very limited subset of patients with rapidly progressive renal failure who remain normotensive and develop ANCA-positive crescentic glomerulonephritis. We report a case of normotensive acute renal failure secondary to anti-MPO antibody-associated crescentic glomerulonephritis in a patient with diffuse systemic sclerosis. She was referred to our department with normal blood pressure and no extrarenal clinical manifestation ofvasculitis. She presented with rapidly progressive renal failure, microscopic hematuria and minimal proteinuria. P-ANCA were positive by immunofluorescence, with ELISA-confirmed specificity for myeloperoxidase. Renal biopsy revealed typical features of pauciimmune glomerulonephritis with crescent formation and fibrinoid necrosis. The patient was initially treated with i.v. cyclophosphamide only. Because of ongoing deteriorating renal function, additional treatment with intravenous pulses of methylprednisolone followed by oral prednisone was started and allowed renal function improvement. After 9 months, serum creatinine had almost returned to normal level with minimal proteinuria, no hematuria and negative ANCA testing. Control kidney biopsy only revealed scar lesions. The association of ANCA-positive crescentic glomerulonephritis and systemic sclerosis is a very rare event. Treatment with intravenous cyclophosphamide and corticosteroids allows rapid and long-term improvement of renal function. The onset of typical scleroderma renal crisis triggered by high-dose corticosteroids is unlikely but requires a close follow-up of patients with overlapping systemic sclerosis. Diagnosis and treatment are discussed and previously published cases are reviewed.
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PMID:ANCA-related crescentic glomerulonephritis in systemic sclerosis: revisiting the "normotensive scleroderma renal crisis". 1791 19

An insidious onset of unexplained fever, weight loss, skin lesions, abdominal pain, and musculoskeletal pain should suggest the diagnosis of polyarteritis nodosa (PAN). However, familial Mediterranean fever (FMF) with protracted febrile myalgia (PFM) should be kept in mind in the differential diagnosis. In this report, 6 cases of PFM mimicking PAN are described. Patients presented with severe muscle and abdominal pain lasting longer than 4 weeks. Their common medical history included recurrent febrile abdominal pain or arthritis. Physical examination revealed hypertension together with severe muscle tenderness. Laboratory examination revealed high acute phase reactants, negative p-ANCA, normal creatine kinase, and complement levels. Duplex abdominal ultrasonography was normal. Four of 6 patients were hospitalized with initial diagnoses of PAN. Renal and mesenteric angiography performed in 1 patient was normal. Steroid therapy controlled all the severe symptoms including hypertension in all of the cases.FMF with PFM is important in the differential diagnosis of patients with suspected vasculitis especially when myalgia is present. Hypertension may be present as a result of sympathetic discharge because of severe myalgia. Because PFM rapidly responds to a short course of corticosteroids, a rapid diagnosis of PFM in FMF patients can reduce unnecessary workup and decrease the time patients have to suffer.
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PMID:Protracted febrile myalgia mimicking polyarteritis nodosa. 1863 21

A 72-year-old woman was admitted to our hospital because of massive proteinuria of 2.2 g/day. She had seen a general practitioner for management of Basedow disease, diabetes mellitus and hypertension for 24 years. On admission, she complained of anorexia and nausea. Laboratory data showed serum creatinine of 3.62 mg/dL and MPO-ANCA of 68 EU. Renal biopsy revealed crescentic glomerulonephritis complicated with membranous nephropathy. Thiamazole (MMI), which was being given for Basedow disease for years, was withdrawn on the suspicion as a cause of MPO-ANCA. Three years after the withdrawal of MMI, renal failure slowly progressed to the end-stage, while MPO-ANCA was negative. She was introduced onto hemodialysis. At that time, MPO-ANCA became positive again, the titer being 12.9 EU. Therefore, we suspected a relapse of ANCA-related vasculitis and performed steroid pulse therapy on the patient with methylprednisolone at 0.5 g/day for 3 days. On hospital day 14, MPO-ANCA became negative. On day 25, however, severe acute pancreatitis developed and a pancreatic tumor lesion was found on CT. In spite of amelioration of the pancreatitis by medical treatment, the pancreatic tumor lesion did not show any significant change. On day 48, she died of multiple organ failure. Autopsy showed a mucinous cyst adenoma of pancreas and necrotizing pancreatitis. We suspected steroid therapy as the cause of the pancreatic lesions.
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PMID:[MPO-ANCA related vasculitis complicating mucinous cystadenoma of the pancreas and severe acute pancreatitis after steroid pulse therapy: a case report]. 1906 54

The patient (woman), 54 years of age, treated for p-ANCA positive vasculitis, hypertension, and thyroid gland hypofunction was referred to our Department due to the decrease of the visual acuity of her left eye. Since the year 2003, she was followed-up in the Department of Neurology due to the idiopathic intracranial hypertension (the opening pressure of the intracranial liquid was 480 -500 mm of H2O column), treated by acetazolamide tablets (Diluran) 1/2 tablet once daily. She was followed-up for the bilateral persistent papilledema by the local ophthalmologist. Due to the progression of the visual acuity loss the decompression (fenestration) of the optic nerve sheath was performed, and the neurosurgeon recommended the lumbo-peritoneal shunting. Adequate indication and choice of the surgical treatment helped to stabilize visual functions.
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PMID:[Benefit of the surgical treatment of the idiopathic intracranial hypertension--a case report]. 2005 24

A 86-year-old man had been treated for hypertension, diabetes mellitus (DM), and dyslipidemia in Nihonkoukan Hospital. His renal function was within the normal range in August 2007. He showed common cold-like symptoms, which were not improved by anti-inflammatory drugs in December 2007. He was admitted to our hospital because of renal failure, urine protein and urine occult blood. He was also positive for anti-myeloperoxidase antibody (MPO-ANCA; 129 IU/mL). A renal biopsy revealed idiopathic crescentic glomerulonephritis of the pauci immune type. Considering his advanced age and DM, he was treated with the low dose of 20 mg/day of prednisolone. Although his symptoms, such as low grade fever and general fatigue, were improved after steroid therapy, renal failure accelerated, necessitating hemodialysis (HD), and insulin administration was needed for his DM. Subsequently, an AV fistule operation for HD was performed. Prednisolone was tapered to 17.5 mg/day after 4 weeks, and his MPO-ANCA titer decreased to 87 IU/mL. After steroid treatment and HD, his condition gradually recovered and he was discharged on March 5, 2008. Following about 6 months of treatment with prednisolone (3.5 months after HD administration), his renal function gradually recovered, allowing the discontinuation of HD. High-dose steroid therapy is very effective for ANCA-related glomerulonephritis. However, there is a high risk of infection, especially in aged and DM patients. Low-dose steroid therapy (PSL 20 mg/day) is safe and effective in such high-risk patients and in some cases, they can be released from HD.
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PMID:[ANCA-related glomerulonephritis in an aged patient with diabetes mellitus successfully released from hemodialysis by low dose steroid therapy: a case report]. 2168 87


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