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Diseases of an autoimmune nature are well recognized in association with primary biliary cirrhosis. Although autoimmune thyroiditis and many rheumatological conditions are well described in primary biliary cirrhosis, autoimmune haematological diseases have been less well reported. We report on a 66 year old North American Indian man with coincident primary biliary cirrhosis and warm antibody haemolytic anaemia. This case report supports the suggestion of an association between autoimmune haemolytic anaemia and primary biliary cirrhosis.

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A number of clinical laboratory and biopsy-derived parameters were assessed for their prognostic significance in the short (24 months), intermediate (60 months) and long terms in 45 patients (43 female, 2 male) with diffuse proliferative lupus glomerulonephritis (DPGN). The factors evaluated were serum creatinine (SCr) and urinary protein at time of biopsy, initial dose of prednisone and immunosuppressive after biopsy, activity index (AI), chronicity index (CI), their individual components, extent of extraglomerular (tubulo-interstitial) immune deposits (EGD) and mean number of intraglomerular monocytes per glomerulus (NSE index). Using proportional hazards analysis to evaluate the parameters, SCr (P = 0.003), AI (P = 0.005) and NSE index (P = 0.038) were shown to be significant predictors of outcome when all variables except the components of AI and CI were considered. When AI and CI were omitted but their components included, SCr (P = 0.0005), NSE index (P = 0.024), extent of karyorrhexis (P = 0.035) and glomerulosclerosis (P = 0.033) were then demonstrated to be significant prognostic factors of DPGN. The results suggest that intraglomerular monocyte infiltration has a protective effect and confirm that AI index is a relatively powerful predictor of outcome. Histologic and nonhistologic biopsy factors contribute significant additional prognostic information to that provided by SCr.

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Herein is reported the case of a man who has had a recurrence of Goodpasture's syndrome following a five-year remission. The patient presented initially in 1977 at the age of 28 with Goodpasture's syndrome manifested by pulmonary hemorrhage without clinical evidence of renal disease, and positive antiglomerular basement membrane antibody. Following treatment with corticosteroids, remission occurred and the serum antiglomerular basement membrane antibody became negative. In 1983, he experienced a relapse with the reappearance of serum antiglomerular basement membrane antibody, the development of severe life-threatening intrapulmonary hemorrhage, and hematuria. This case illustrates that life-threatening relapse may occur in Goodpasture's syndrome despite a prolonged remission and the disappearance of detectable antiglomerular basement membrane antibody in the circulation.

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Previous studies of IgA nephropathy have demonstrated a number of prognostically significant clinical and pathological factors in groups of patients with the full histological spectrum of the disease. Whether these factors can be applied to a group of IgA nephropathy patients with disease of moderate degree is unknown. Forty patients (9 females, 31 males) with grade III IgA nephropathy (no more than 10% obsolete glomeruli and little or no interstitial fibrosis) were evaluated with respect to age, sex, degree of proteinuria, history of recurrent gross hematuria, hypertension, extent and type of segmental glomerulosclerosis, demonstration of IgG and/or IgM in deposits, presence of peripheral capillary deposits, whether or not there were crescents, and extent of vascular sclerosis. The mean age was 29.6 +/- (SD) 13.1 years. Sixteen patients presented with recurrent gross hematuria, and 24 had microscopic hematuria and proteinuria as the initial manifestation. Hypertension was seen in 5 patients. The mean serum creatinine concentration was 1.09 +/- 0.47 mg/dl (96.4 +/- 41.5 mumol/l), and the mean 24-hour urinary protein was 1.5 +/- 1.3 g. Nine patients had proteinuria greater than or equal to 2.0 g/24 h. Thirty-two patients demonstrated segmental glomerulosclerosis in their biopsies, 13 of which had more than 10% of the glomeruli involved. Seven patients developed established renal failure (Cr greater than or equal to 2.0 mg/dl; 176.8 mumol/l). The 60-and 100-month renal survival rates were 96 and 52%. Life table analysis disclosed that only the degree of proteinuria (greater than or equal to 2.0 g/24 h; p less than 0.05) and the extent of segmental glomerulosclerosis (p less than 0.025) were of prognostic significance.(ABSTRACT TRUNCATED AT 250 WORDS)

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Clinical, laboratory and pathological factors in 35 females with diffuse proliferative lupus glomerulonephritis were analyzed to determine the prognostic significance of the individual variables. The clinical and laboratory variables were age, serum creatinine (Cr), serum C3, serum C4 and proteinuria at the time of biopsy while the biopsy ones included intraglomerular monocytic infiltration (NSE index), total glomerular deposits, extent of subendothelial deposits, extent of extraglomerular deposits, tubulo-interstitial inflammation, relative tubulo-interstitial volume and total pathologic score. Standard morphometric and counting procedures were used to determine the levels of all pathologic variables but pathologic score and extra glomerular deposits where grading estimates were done. Survival curves were determined by the life table method. Logrank and chi-square tests were used to establish levels of statistical significance. Seven patients developed established renal failure (Cr greater than or equal to 2.0 on two or more occasions at least 3 months apart) and nine showed significant deterioration of renal function (decrease in CrCl of 25% or more in between biopsy and last follow-up visit or an increase in serum Cr of 0.4 mg/dl or more over the follow-up period). The 5-year renal survival rate (absence of established renal failure) for the whole group was 77%. Serum Cr (p less than .005) and extent of extraglomerular deposits (p less than .025) were shown to be significant prognostic factors for renal survival. Of the seven patients who developed renal failure none had an NSE index greater than 3.0 and one had a C3 greater than or equal to 45 mg/dl. Statistically these factors were weak prognostic indicators (0.5 less than p less than .1). Multivariate analysis demonstrated that the extraglomerular deposit factor contributed significant additional prognostic information to that provided by Cr. Although not important as a prognostic factor on its own, the NSE index significantly improved the prognostic performance of serum Cr. The product of the NSE index and serum C3 proved to be a strong prognostic factor (p less than .005).

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Focal proliferative lupus glomerulonephritis has traditionally been considered to be more benign than the diffuse form. A recent study of lupus nephritis using the W.H.O. classification demonstrated no difference in 4-year survival between those with focal and those with diffuse disease. Because of this development, a comparative clinicopathologic study of 15 patients with focal proliferative lupus glomerulonephritis was done using the W.H.O. classification. Generally, patients with focal proliferative lupus glomerulonephritis presented with milder renal disease with respect to proteinuria and renal insufficiency. Involvement of the central nervous system was more prominent in focal proliferative lupus glomerulonephritis. Therapy for both types of disease was similar. Mean duration of renal disease was 48 months for focal and 50.7 months for diffuse disease. Three patients with focal proliferative lupus glomerulonephritis and two with diffuse proliferative lupus glomerulonephritis were dead at the end of the follow-up period. Established renal failure was present in one patient with focal disease and two with diffuse disease. Deterioration of renal function was noted in two patients with focal proliferative lupus glomerulonephritis and five with diffuse proliferative lupus glomerulonephritis at the end of the follow-up period. No statistically significant differences in cumulative five-year survival rates (focal = 0.751; diffuse = 0.858), cumulative five-year renal survival rates (focal = 1.00; diffuse = 0.846), deterioration of renal function and quantitative proteinuria at the end of the follow-up period were noted. although qualitatively milder, the focal form of renal disease followed a course similar to that of the diffuse type.

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Renal failure insidiously developed in three patients treated with a combination of hydrochlorothiazide and triamterene five to 10 weeks after commencing therapy. All had normal renal function prior to therapy and none had preexisting renal disease. Two of the patients had eosinophilia and two had a fever. One patient was oliguric. Renal biopsy demonstrated acute interstitial nephritis histologically. Direct immunofluorescence did not disclose evidence of either immune complex or antitubular basement membrane antibody deposition. Withdrawal of the drug led to remarkable improvement with renal function returning to normal in two patients and near normal in the third. The sequence of events and the histologic findings strongly suggest that the acute interstitial nephritis was due to a drug-induced hypersensitivity reaction. Although hydrochlorothiazide appears to be the drug responsible for the reaction, certain observations suggest a possible potentiating role for triamterene.

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5 patients are described who developed severe osteomalacia with spontaneous fractures after 2-4 years on dialysis. Phosphate control, vitamin D2 therapy and parathyroidectomy were ineffective. These individuals showed a hypercalcemic tendency but little histologic or radiographic evidence of osteitis fibrosa. After parathyroidectomy, the hypercalcemic tendency remained and bone biopsy revealed gross osteomalacia. A 6- to 12-month therapeutic trial with 1,25-dihydroxycholecalciferol (1,25[OH]2D3) in 3 did not arrest skeletal deterioration. 4 subsequently developed dialysis encephalopathy. These patients appear to have a unique mineralizing defect unresponsive to 1,25(OH)2D3. This "dialysis osteomalacic syndrome" may result from toxic substances associated with uremia or the hemodialysis regimen.

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Six long-term hemodialysis patients with progressive skeletal deterioration during long-term pharmacologic vitamin D2 therapy were treated for six to 12 months with oral 1,25-dihydroxycholecalciferol (1,25-(OH)2D3) to determine its therapeutic effectiveness in vitamin D2-unresponsive osteodystrophy. On bone biopsy, three of the patients had severe osteomalacia and three showed predominant osteitis fibrosa. Previous therapies, including phosphate binders and dialysis schedules, were maintained. The three patients with osteomalacia and the two with osteitis fibrosa showed clinical deterioration. There was no significant change in serum calcium, phosphate, alkaline phosphatase, bone densitometry, immunoreactive parathyroid hormone levels or bone histology. Roentgenograms showed multiple new fractures of ribs and femoral necks in the patients with osteomalacia and increased bone resorption in two of three patients with osteitis fibrosa. 1,25-(OH)2D3 dosage had to be decreased in all patients because of hypercalcemia with a mean tolerated dose of 0.22 microgram/day. In these patients, 1,25-(OH)2D3 was not effective therapy for progressive osteodystrophy unresponsive to pharmacologic vitamin D2.

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A 28-year-old man developed recurrent hemoptyses, breathlessness, anemia, and bilateral pulmonary infiltrates after mild smoke inhalation. He had no laboratory evidence of kidney involvement. Transbronchial lung biopsy showed erythrocytes, iron-containing macrophages within alveolar spaces, normal basement membranes, and strongly positive linear staining of alveolar septa for immunoglobulin G (IgG). Serum antiglomerular basement-membrane antibody was strongly positive by radioimmunoassay. Kidney biopsy showed normal findings by light and electron microscopy but strongly positive linear staining of glomerular capillaries for IgG. Follow-up 9 months later while the patient was taking prednisone revealed no clinical evidence of pulmonary or renal disease. This case shows that immunopathologic study of transbronchial lung biopsies is helpful in differentiating between Goodpasture's syndrome and idiopathic pulmonary hemosiderosis, while the absence of clinical and microscopic evidence of kidney disease does not exclude Goodpasture's syndrome.

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The yellow nail syndrome, a combination of yellow discolouration of and dystrophic changes in the nails, pleural effusions and lymphedema, is thought to be relatively rare; to date 44 cases have been reported. Of a further three patients with this syndrome, one had all three features, one had the yellow nails alone and the other had pleural effusions and lymphedema without classic nail changes. Each had recurrent lower respiratory tract infections; and of all 47, chronic pulmonary infections occurred in approximately one quarter and were frequently associated with chronic sinus infections. The underlying abnormality is presumed to be a congenital defect of the lymphatics, but so far this has not been demonstrated to be the cause of the nail changes, the pathogenesis of which remains obscure.

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