• Title/Summary/Keyword: Goodpasture's syndrome

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A Case of Goodpasture's Syndrome with Normal Renal Function (정상 신기능을 보인 Goodpasture 증후군 1예)

  • Kim, Sung Kyoung;Lee, Jin Kook;Joo, Kyeo Rae;Hur, Sung Eun;Kim, Jeong Hoon;Kim, Young Kyoon;Lee, Sook Young;Kim, Seung Joon;Kim, Chi Hong;Song, So Hyang;Park, Sung Hark;Choi, Young Jin
    • Tuberculosis and Respiratory Diseases
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    • v.59 no.1
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    • pp.86-92
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    • 2005
  • 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.

A Case of Goodpasture's Syndrome Combined with Crohn's Disease (크론씨 병에 동반된 Goodpasture 증후군 1예)

  • Kim, Ji-Yon;Bae, Jun-Yong;Jung, Eun-Jung;Kim, Yang-Ki;Lee, Young Mok;Kim, Ki-Up;Uh, Soo-taek;Hwang, Jung-Hwa;Jin, So-Young;Lee, Dong-Wha
    • Tuberculosis and Respiratory Diseases
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    • v.61 no.4
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    • pp.384-388
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    • 2006
  • 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.

A Case of Microscopic Polyangiitis with Diffuse Alveolar Hemorrhage (폐출혈을 동반한 현미경적 다발혈관염 1예)

  • Lee, Sang-Jin;Lee, Jae-Woung;Kim, Hye-Jin;Shin, Kyeong-Cheol;Chung, Jin-Hong;Lee, Kwan-Ho;Park, Hye-Jung
    • Journal of Yeungnam Medical Science
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    • v.21 no.1
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    • pp.101-107
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    • 2004
  • Diffuse alveolar hemorrhage is a rare but serious and frequently life-threatening complication of a variety of conditions. The first goal in the management of patients with diffuse alveolar hemorrhage is to achieve or preserve stability of the respiratory status. Subsequently, the differential diagnosis is aimed at the identification of a remediable cause of the alveolar hemorrhage. The most common causes of diffuse alveolar hemorrhage with glomerulonephritis are microscopic polyangiitis and Wegener's granulomatosis, followed by Goodpasture syndrome and systemic lupus erythematosus. Microscopic polyangiitis (MPA) is a distinct systemic small vessle vasculitis affecting small sized vessels with few or no immune deposits and with no granulomatosus inflammation. The disease may involve multiple organs such as kidney, lung, skin, joint, muscle, gastrointestinal tract, eye, and nervous system. MPA is strongly associated with antineutrophil cytoplasmic autoantibody (ANCA) that is a useful serological diagnostic marker for the most common form of necrotizing vasculitis. Our report concerns a case of microscopic polyangiitis with diffuse alveolar hemorrhage in a 54-year-old man. He was admitted to our hospital due to dyspnea upon exertion and recurrent hemoptysis. Laboratory findings showed hematuria, proteinuria and deterioration of renal function. In the chest CT scan, diffuse ground glass appearance was seen in both lower lungs. A lung biopsy revealed small vessel vasculitis with intraalveolar hemorrhage and showed a positive reaction to against perinuclear ANCA. The patient was treated with prednisolone and cyclophosphamide. Chest infiltration decreased and hemoptysis and hypoxia improved. He is still being followed up in our hospital with a low dose of prednisolone.

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