Cases reported "Sjogren's Syndrome"

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1/19. Mesangial nephropathy in sjogren's syndrome.

    We describe a 36-year-old woman with Primary sjogren's syndrome (PSS). purpura, corneal perforation, metabolic acidosis, decreased glomerular filtration, hypokalemia, hyposthenuria, and polyuria were present. Chronic renal insufficiency and renal tubular acidosis type I were diagnosed. kidney biopsy revealed mesangial glomerulonephritis, interstitial nephritis, and tubular atrophy. Replacement treatment with saliva, tears, and potassium citrate was started. She was given prednisone and cyclophosphamide. This would be the first description of PSS, mesangial glomerulonephritis, and chronic renal insufficiency.
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ranking = 1
keywords = glomerulonephritis
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2/19. Clinically significant and biopsy-documented renal involvement in primary Sjogren syndrome.

    Clinically significant renal involvement in patients with primary Sjogren syndrome (pSS) has been described previously only in isolated case reports. The prevalence and significance of the 2 described syndromes, interstitial nephritis (IN) and glomerulonephritis (GMN), are not well known. In a cohort of 471 patients with pSS who were followed for a mean of 10 years, 20 patients (4.2%) developed overt renal disease. Eighteen patients underwent a percutaneous renal biopsy; 2 patients declined. Ten patients had IN, 8 patients had GMN, and 2 patients presented with both entities. Glomerular histology disclosed changes compatible with membranoproliferative GMN in 5 patients and mesangial proliferative GMN in 4 patients. patients with IN had a younger disease onset compared with patients with GMN (mean, 36.8 compared with 46.0 yr, p 5 0.063). patients with GMN had longer disease duration compared with patients with IN (mean, 2.2 compared with 8.0 yr, p 5 0.001). The majority of patients with GMN (80%) had mixed monoclonal cryoglobulinemia IgMk (type II) and lower complement c4 levels. Two patients (both with GMN) developed chronic renal failure requiring hemodialysis. overall, clinically significant renal involvement is infrequent in pSS. IN occurs early in the disease process, while GMN is a late sequela and may have a less favorable prognosis.
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ranking = 0.92490766305515
keywords = glomerulonephritis, membranoproliferative
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3/19. Antineutrophil cytoplasmic autoantibody-associated vasculitis presenting as sjogren's syndrome.

    A 63-year-old woman, in whom a diagnosis of sjogren's syndrome was initially made, proved to have systemic vasculitis with salivary gland involvement and necrotizing and crescentic glomerulonephritis. Antineutrophil cytoplasmic autoantibodies (ANCA) against myeloperoxidase were positive. ANCA-associated vasculitis should be considered in the differential diagnosis of sjogren's syndrome. A positive finding on immunoassay for ANCA against myeloperoxidase or proteinase 3 may help establish the diagnosis.
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ranking = 0.5
keywords = glomerulonephritis
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4/19. Immune complex glomerulonephritis in sicca syndrome.

    In three patients with the sicca syndrome (sjogren's syndrome), who were followed for one to seven years, glomerulonephritis developed. None of these patients fulfilled the diagnostic criteria for systemic lupus erythematosus. All of these patients had circulating immune complexes as detected by the Clq binding assay. Glomerular histology by light and electron microscopy revealed changes compatible with membranoproliferative glomerulonephritis in two of the patients and membranous glomerulonephritis in the third. All patients showed rapid improvement in renal function following moderate doses of corticosteroids. In addition, the treatment decreased the level of circulating immune complexes in two patients who were followed for a sufficient period of time.
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ranking = 4.262434702965
keywords = glomerulonephritis, membranoproliferative, membranoproliferative glomerulonephritis
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5/19. Primary sjogren's syndrome complicated with cryoglobulinemic glomerulonephritis, myocarditis, and multi-organ involvement.

    glomerulonephritis in primary sjogren's syndrome is rarely reported. Cryoglobulinemic glomerulonephritis with the presence of cryoglobulin deposition in the glomerular capillary lumen in primary sjogren's syndrome is extremely rare. A 51-year-old woman with primary sjogren's syndrome for > 10 years complained of fever, hypertension, and proteinuria. In addition, novel manifestations, including myocarditis with heart failure, pericardial effusion, and polyneuropathy (sensory motor neuropathy) were also noted. cryoglobulinemia test was positive, and kidney biopsy results were consistent with cryoglobulinemic glomerulonephritis. There were no symptoms associated with systemic lupus erythematosus or other connective tissue disease. Treatment with monthly methylprednisolone and cyclophosphamide pulse therapy for 6 months resulted in resolution of proteinuria, heart failure, and neurologic symptoms.
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ranking = 3
keywords = glomerulonephritis
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6/19. Acute renal failure due to mesangial proliferative glomerulonephritis in a pregnant woman with primary sjogren's syndrome.

    The most common form of renal involvement in sjogren's syndrome (SS) is tubulointerstitial nephritis. Renal dysfunction is usually mild and subclinical. glomerulonephritis (GMN) is rare in patients with SS. We report a 28-year-old multigravida patient with primary sjogren's syndrome (pSS) and associated manifestations, who presented with acute renal failure in the 20th week of her fifth pregnancy. The complaints and clinical findings, positive Schirmer's test, findings of dry eye on ophthalmologic examination, and the salivary gland biopsy were compatible with SS. The patient exhibited no other clinical or laboratory findings indicative of other collagenous disease and/or rheumatoid arthritis. She refused renal biopsy, hesitating for fear of fetal loss; thus, based on the clinical and laboratory findings indicating rapidly progressive GMN and vasculitis, prednisolone, plasmapheresis, and one dose of cyclophosphamide were administered during the pregnancy. Hemodialysis five times weekly was performed. At the 28th week of gestation, she underwent a cesarean section due to early rupture of membranes and fetal distress. A healthy male boy was delivered. The renal biopsy performed 2 weeks after labor revealed mesangial proliferative glomerulonephritis. After the fourth cyclophosphamide treatment, her urinary output increased and she was discharged from the hemodialysis program. She remains in follow-up at our outpatient clinic free of hemodialysis for 4 months. This is the first report of mesangial proliferative GMN requiring dialysis in a pregnant pSS patient that has featured good maternal and fetal outcomes.
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ranking = 2.5
keywords = glomerulonephritis
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7/19. mixed connective tissue disease associated with MPO-ANCA-positive polyangiitis.

    The patient was a 42-year-old woman diagnosed as having MCTD and sjogren's syndrome in 1989, and who was taking oral prednisolone. proteinuria and microscopic hematuria were pointed out for the first time in December 2004. She was referred to our hospital because of massive hemoptysis. Advanced renal failure, anemia and pulmonary alveolar hemorrhage were diagnosed on admission. She was positive for serum MPO-ANCA. The patient was started on a therapy that included steroids, cyclophosphamide and plasmapheresis. However, her respiratory condition was untreatable and she died on the 16th day of hospitalization. The autopsy revealed alveolar hemorrhage in the lungs and crescentic glomerulonephritis. This patient was considered as a rare case of MCTD associated with MPO-ANCA-positive microscopic polyangiitis.
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ranking = 0.5
keywords = glomerulonephritis
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8/19. sjogren's syndrome complicated by cryoglobulinaemia and acute renal failure.

    A patient is described with sjogren's syndrome and an IgM-kappa gammopathy. With time cryoglobulins, the cause of acute renal failure, could be detected. The kidney biopsy showed a proliferative glomerulonephritis as is frequently seen with mixed cryoglobulinaemia. plasmapheresis and immunosuppression resulted in long-term improvement of kidney function. The differential diagnosis of acute renal failure of glomerular origin in a patient with sjogren's syndrome is discussed. This case report illustrates once more the consequences of monoclonal B-cell lymphocyte activation in sjogren's syndrome.
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ranking = 0.5
keywords = glomerulonephritis
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9/19. Type III membranoproliferative glomerulonephritis in primary sjogren's syndrome.

    glomerulonephritis is a rare complication of primary sjogren's syndrome. When glomerulonephritis does occur, it is usually due to membranous, focal proliferative, or membranoproliferative lesions. There have been fewer than 20 reported cases of glomerulonephritis in primary sjogren's syndrome, only three of which have been membranoproliferative. We report a case of primary sjogren's syndrome complicated by Type III membranoproliferative glomerulonephritis, a specific type of mesangiocapillary glomerulonephritis never before reported in this setting, and describe a clinical course terminating in rapidly progressive renal failure.
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ranking = 8.6619888409352
keywords = glomerulonephritis, membranoproliferative, membranoproliferative glomerulonephritis
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10/19. Auricular chondritis and diffuse proliferative glomerulonephritis in primary sjogren's syndrome.

    Bilateral auricular inflammation with histological changes of relapsing polychondritis was observed in a female patient with primary sjogren's syndrome. This was accompanied by rapidly progressive renal insufficiency due to diffuse proliferative glomerulonephritis. To our knowledge this is the first well documented case of primary sjogren's syndrome associated with chondritis and glomerulonephritis, further emphasising the wide spectrum of extraglandular manifestations in this autoimmune disorder.
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ranking = 3
keywords = glomerulonephritis
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