A 29-year-old male patient was admitted due to his general weakness and poor oral intake for several months. He was diagnosed as having Crohn disease 16 years ago and total colectomy was performed 10 years ago. On the 3rd day after admission, gross hematuria and sudden hemoptysis combined with diffuse infiltration were noted on chest X-ray. His symptoms and the diffusely increased lung opacities improved with administering high-dose steroid therapy. Later, anti-GBM antibody was found to be positive on the laboratory findings. We report here on a rare case of Goodpsture syndrome combined with prolonged Crohn disease along with a review of literature.
Adult ; Anti-Glomerular Basement Membrane Disease* ; Colectomy ; Crohn Disease* ; Hematuria ; Hemoptysis ; Humans ; Lung ; Male ; Thorax

Anti-glomerular basement membrane disease is a rare autoimmune disease characterized by rapidly progressive renal failure and/or pulmonary hemorrhage. The presence of severe crescentic glomerular inflammation with linear deposition of immunoglobulin G along the glomerular basement membrane is pathognomonic. Because renal function is rapidly and often irretrievably destroyed, many patients require hemodialysis all through their lifetime. We report a case of 33 year(s)-old man who was diagnosed as anti-glomerular basement membrane disease without pulmonary hemorrhage. The patient was treated with pulse methylprednisolone and plasmapheresis followed by oral corticosteroid and cyclophosphamide. His renal function was successfully recovered with early diagnosis and aggressive treatment.
Adrenal Cortex Hormones ; Anti-Glomerular Basement Membrane Disease ; Autoimmune Diseases ; Cyclophosphamide ; Early Diagnosis ; Glomerular Basement Membrane ; Hemorrhage ; Humans ; Immunoglobulin G ; Immunosuppression ; Inflammation ; Methylprednisolone ; Plasmapheresis ; Renal Dialysis ; Renal Insufficiency

Up to 40% of patients with anti-glomerular basement membrane (GBM) disease, which is a rare autoimmune disorder usually manifesting as rapidly progressive glomerulonephritis (RPGN), are positive for circulating anti-neutrophil cytoplasmic antibody (ANCA). Many previous reports showed poor outcomes in these "double-positive" patients. We report a patient with perinuclear (p)-ANCA positive anti-GBM disease who presented with RPGN and required hemodialysis. Plasmapheresis and steroid and cyclophosphamide therapy were initiated following renal biopsy and resulted in normalization of anti-GBM antibody and p-ANCA titers, recovery of renal function, and discontinuation of hemodialysis. This case suggests that aggressive immunosuppression with plasmapheresis in patients who are p-ANCA positive with anti-GBM disease should be considered, even in those with severe renal dysfunction.
Anti-Glomerular Basement Membrane Disease ; Antibodies, Antineutrophil Cytoplasmic ; Autoantibodies ; Basement Membrane ; Biopsy ; Cyclophosphamide ; Glomerulonephritis ; Hemorrhage ; Humans ; Immunosuppression ; Lung Diseases ; Plasmapheresis ; Renal Dialysis

Goodpasture's syndrome is a disease that is characterized by hemoptysis, anemia, and glomerulonephritis with renal failure. Goodpasture reported a case of a young man who expired as a result of a pulmonary hemorrhage and glomerulonephritis at the recovery phase after an influenza infection in 1919. In 1958, Stanton et al. described a combined case of these two diseases as Goodpasture's syndrome. Since then, antiglomerular basement membrane antibody(anti-GBM Ab) has been confirmed to play an important role in the mechanism of this syndrome, and it was reported that this syndrome was an autoimmune disease. The triad of alveolar hemorrhage, glomerulonephritis and circulating anti-GBM Ab forms the basis of a diagnosis of Goodpasture's syndrome. When patients are affected by disease, the relief of symptoms can be accomplished by eliminating the anti-GBM Ab from the circulatory system through hemodialysis, plasmapheresis and immunoabsorption. However, the patients usually die from a massive pulmonary hemorrhage when the diagnosis or treatment is delayed. The incidence of Goodpasture's syndrome is common in the western world, but it is extremely rare in Korea with only five cases being reported. In three of these cases, pulmonary hemorrhage and renal failure was the initial manifestation. Therefore, hemodialysis or plasmapheresis were absolutely essential treatments. We report a case of Goodpasture's syndrome in Korea with a normal renal function.
Anemia ; Anti-Glomerular Basement Membrane Disease* ; Autoimmune Diseases ; Basement Membrane ; Diagnosis ; Glomerulonephritis ; Hemoptysis ; Hemorrhage ; Humans ; Incidence ; Influenza, Human ; Korea ; Plasmapheresis ; Renal Dialysis ; Renal Insufficiency ; Western World

Anti–glomerular basement membrane (GBM) nephritis is characterized by circulating anti-GBM antibodies and crescentic glomerulonephritis (GN) with deposition of IgG along the GBM. In a limited number of cases, glomerular immune complexes have been identified in anti-GBM nephritis. A 38-year-old female presented azotemia, hematuria, and proteinuria without any pulmonary symptoms. A renal biopsy showed crescentic GN with linear IgG deposition along the GBM and mesangial IgA deposition. The patient was diagnosed as concurrent anti-GBM nephritis and IgA nephropathy. Therapies with pulse methylprednisolone and cyclophosphamide administration were effective. Concurrent cases of both anti-GBM nephritis and IgA nephropathy are rare among cases of anti-GBM diseases with deposition of immune complexes. This rare case of concurrent anti-GBM nephritis and IgA nephropathy with literature review is noteworthy.
Adult ; Anti-Glomerular Basement Membrane Disease ; Antibodies ; Antigen-Antibody Complex ; Azotemia ; Basement Membrane ; Biopsy ; Cyclophosphamide ; Female ; Glomerulonephritis ; Glomerulonephritis, IGA ; Hematuria ; Humans ; Immunoglobulin A ; Immunoglobulin G ; Methylprednisolone ; Nephritis ; Proteinuria

Both anti-glomerular basement membrane (GBM) disease and the anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are common causes of pulmonary-renal syndrome. Organizing pneumonia (OP), a special pattern of interstitial lung disease, is extremely rare either in AAV or anti-GBM disease. We report an old woman presented with OP on a background of co-presentation with both ANCA and anti-GBM antibodies.
Female ; Humans ; Antibodies, Antineutrophil Cytoplasmic ; Organizing Pneumonia ; Autoantibodies ; Glomerulonephritis ; Anti-Glomerular Basement Membrane Disease ; Pneumonia ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/complications*

Goodpasture's syndrome (GS) is characterized by lung hemorrhage and glomerulonephritis and caused by autoimmune reaction between anti-glomerular basement membrane (GBM) autoantibodies and the alpha 3 (type IV) collagen chain. Some reports suggested that patients with anti-GBM autoantibody could be related with other autoimmune diseases including Graves' disease. We report a case of 14-year-old girl with Graves' disease treated with PTU for 4 years, who was admitted because of hemoptysis and dyspnea. Laboratory values included a serum creatinine value of 0.7 mg/dL, BUN 22 mg/dL, hemoglobin 3.9 g/mm3 and albumin 3.2 mg/dL. The thyroid function tests showed normal serum T3 and free T4, suppressed TSH, and elevated thyroglobulin Ab and TSH-R-Ab levels. Urinalysis showed 2+ for protein and many dysmorphic RBC/HPF. Both anti-GBM Ab and pANCA were positive serologically. In renal biopsy, the glomeruli showed mesangial proliferation and crescent formation with linear deposits of IgG along the GBM. This case is to represent the GS of patient with Graves' disease referring to medical documents.
Adolescent ; Anti-Glomerular Basement Membrane Disease* ; Autoantibodies ; Autoimmune Diseases ; Basement Membrane ; Biopsy ; Collagen ; Creatinine ; Dyspnea ; Female ; Glomerulonephritis ; Graves Disease* ; Hemoptysis ; Hemorrhage ; Humans ; Immunoglobulin G ; Lung ; Methylprednisolone ; Thyroglobulin ; Thyroid Function Tests ; Urinalysis

Wilson's disease is an autosomal recessive disorder of copper metabolism in individuals with mutant ATP7B genes. Impairment of normal excretion of hepatic copper results in toxic accumulation of the metal in liver, brain and other organs. Clinical manifestations include hepatic, neurologic or psychiatric disturbances. Penicillamine, as a chelator of copper, is the drug of choice in the treatment of Wilson's disease but after treatment of penicillamine, granulocytopenia, thrombocytopenia, the nephrotic syndrome, Goodpasture's syndrome, pemphigus vulgaris or pleural effusion may supervene. We report a case of macromastia with multiple fibroadenomas in a patient who was treated with penicillamine for Wilson's disease.
Agranulocytosis ; Anti-Glomerular Basement Membrane Disease ; Brain ; Copper ; Fibroadenoma* ; Hepatolenticular Degeneration* ; Humans ; Liver ; Metabolism ; Nephrotic Syndrome ; Pemphigus ; Penicillamine ; Pleural Effusion ; Thrombocytopenia

Transplant glomerulopathy (TGP) is specified as thickening of capillary wall of glomerulus and clinically presented with proteinuria and progressive graft dysfunction. In contrast, crescent formation represents an extracapillary proliferative glomerular change and is clinically presented with rapidly progressive renal failure. Previously, in transplant kidneys, crescent formation was reported only in anti-GBM disease and ANCA- associated vasculitis. Here we report a case with a very unusual combination of transplant glomerulopathy and crescent formation. Ten years after the renal transplantation the patient was admitted due to proteinuria and progressive azotemia. Although his underlying renal disease was IgA nephropathy, the transplant kidney biopsy revealed typical findings of transplant glomerulopathy without specific immune deposits, but with extensive cellular crescents. Methylprednisolone pulse therapy was not successful, and he was switched to maintenance hemodialysis.
Anti-Glomerular Basement Membrane Disease ; Azotemia ; Biopsy ; Capillaries ; Glomerulonephritis, IGA ; Humans ; Kidney ; Kidney Transplantation ; Methylprednisolone ; Proteinuria ; Renal Dialysis ; Renal Insufficiency ; Transplants ; Vasculitis

OBJECTIVETo investigate the clinical and pathologic characteristics of anti-glomerular basement membrane(GBM) disease with normal renal function.

METHODSThe clinical and pathologic data of 6 patients with anti-GBM disease and normal renal function in Peking Union Medical College Hospital were reviewed retrospectively. Furthermore, 29 patients with anti-GBM disease and impaired renal function in the same period in the same hospital were enrolled as the control group. Factors that may influence the prognosis were analyzed.

RESULTSSix (17.1%) of all 35 patients maintained normal renal function for 12-133 months during follow-up. Five patients had microhematuria and proteinuria, one had pulmonary hemorrhage only, and three manifested as Goodpasture syndrome. Renal biopsies from 4 patients revealed linear deposition of IgG 2+-3+ along the glomerular capillary walls by immunofluorescence. As shown by normal light microscopy, mild mesangial proliferation and crescentic glomerulonephritis with a large amount of fibrinoid necrosis of glomerular capillary walls were observed in different patients; however, most pathological changes were mild. Five of these six patients were treated with immunosuppressive drugs and/or plasma exchange. Compared with the control group, the 6 patients with normal renal function had significantly higher hemoglobin[(77.97±20.62 vs.(99.67±19.80 g/L P=0.024], lower titers of anti-GBM antibody[(224.34 ± 145.79 vs.(80.23 ± 85.73 EU/ml P=0.027], and lower ratio of glomeruli with crescents[(0.58±0.29 vs.(0.17±0.27 ,P=0.005]. These 6 patients with normal renal function were followed up for 12-133 months, among whom 4 patients achieved complete remission and 2 had mild proteinuria and microhematuria.

CONCLUSIONAnti-GBM disease with normal renal function is not uncommon. Most patients have mild pathologic changes and good prognosis.

Adult ; Anti-Glomerular Basement Membrane Disease ; pathology ; physiopathology ; Female ; Follow-Up Studies ; Humans ; Kidney ; physiopathology ; Male ; Middle Aged ; Prognosis ; Retrospective Studies