Goodpasture's syndrome

Goodpasture s syndrome (GS) is an auto-immune disease that is part of the pulmonary-renal syndrome spectrum. It is characterized by the linear deposition of anti-glomerular basement membrane antibodies (anti-GBM) in glomerular and alveolar basement membrane resulting in alveolar hemorrhage and progressive glomerulonephritis. An early diagnosis is important to decrease clinical morbidity. In the present work we illustrate a GS case initially diagnosed as Wegener s granulomatosis. The patient showed favorable clinical evolution with corticosteroid therapy associated with plasmapheresis and cyclophosphamide.     

Goodpasture's Syndrome

Summary: Nine cases of Goodpasture's syndrome are reviewed. There has been an apparent change in some aspects of this syndrome since earlier reports. In particular there is less male predominance, a longer survival time and a greater chance of recovery. Various forms of therapy are discussed. In seven treated cases with serial renal biopsy or post mortem specimens, semi-quantitative analysis indicated a lessening of disease activity and a tendency towards histological healing.     

Chronic recurrent Goodpasture's syndrome

A distinctive case of Goodpasture's syndrome characterized by multiple recurrences occurring over 14 years was observed. Each recurrence followed cessation of prednisone and azathioprine therapy, and reinstitution of this therapy resulted in prompt remission. Initially, both glomerulonephritis and hemoptysis were prominent clinical features. In recent years, hemoptysis has been the predominant clinical problem. This and other cases suggest that Goodpasture's syndrome may comprise a wider disease spectrum than generally appreciated.     

Goodpasture's Syndrome in Twins

ABSTRACT. Goodpasture's syndrome occurred within 4 months of each other in identical twin sisters aged 12. In one of them the pulmonary haemorrhages were successfully treated with plasmapheresis. Hereditary as well as environmental factors may contribute to the development of this uncommon disorder.     

Monitoring intrapulmonary hemorrhage in Goodpasture's syndrome

Diffusing capacity of carbon monoxide (Dco) was measured frequently in a patient with recurrent intrapulmonary hemorrhage secondary to Goodpasture's syndrome. This simple test was found to be a sensitive and useful indicator of the presence or absence of recurrent intrapulmonary hemorrhage. As this is now the major cause of mortality in this disease, we recommended that frequent measurements of the Dco be an important part of its management.     

The Many Faces of Goodpasture's Syndrome

ABSTRACT Four cases of antiglomerular basement membrane (antiGBM) antibody mediated disease with unusual features are presented. Lung involvement was absent in one patient whereas the other 3 had Goodpasture's syndrome. Recognition of the nature of the disease was delayed in all cases, due to occurrence during pregnancy in one case and due to the indolent course of the renal injury in the other 3 cases. The therapeutic approach to these variant forms of antiGBM antibody mediated disease is discussed.     

Goodpasture's syndrome with normal renal function

Two cases of Goodpasture's syndrome with severe pulmonary haemorrhage and normal renal function are described. In spite of minor (patient 2) or even no (patient 1) biological or light microscopic signs of glomerulonephritis, immunofluorescence of immunoglobulin G (IgG) was strongly positive in a linear fashion along the glomerular basement membranes in both patients. We suggest that renal biopsy in patients with apparently idiopathic pulmonary haemorrhage may lead to an early diagnosis of Goodpasture's syndrome. It is not possible in this disease to recognize on presentation those patients who will remit spontaneously and those who will undergo severe disease. The deadly evolution for patient 1 and some cases in the literature lend support to the notion that cytostatics and plasma exchange must be added to corticosteroids, even if pulmonary haemorrhage is not active and renal function is normal at the time.     

Immunology of the Goodpasture's syndrome]

About 5% of all human glomerulonephritides are induced by basal membrane antibodies. Immunohistologically they are characterized by a linear fluorescence along the basal membrane of the glomerulum, when cuts of the kidneys of bioptic material are incubated with fluorescent anti-human-IgG-serum. Basal membrane antibodies circulating in the serum as well as antibodies eluted from renal homogenates are nephritogenic. Also recidivations of nephritis on renal homografts confirm the pathogenic significance. Basal membrane antibodies of patients with rapidly progressing glomerulonephritis seem to differ only by a non-existing cross-reaction to the antigen of the basal membrane of the lungs from Goodpasture basal membrane antibodies. Only a glomerulonephritis with pulmonary haemorrhage induced by basal membrane antibodies should be called Goodpasture syndrome.     

Goodpasture's syndrome associated with pulmonary eosinophilic vasculitis

Lung hemorrhage and antiglomerular basement membrane (anti-GBM) antibody mediated nephritis define Goodpasture's syndrome. We present the case of a 19-year-old Caucasian woman with unique clinical findings of Goodpasture's syndrome. Our patient initially presented with leukocytoclastic vasculitis of the skin followed by the development of nephritis and lung hemorrhage. An open lung biopsy done prior to diagnosing anti-GBM antibody disease demonstrated an intense eosinophilic vasculitis. Skin vasculitis has only been rarely reported, and to our knowledge this is the first reported case of pulmonary eosinophilic vasculitis associated with Goodpasture's syndrome.     

Goodpasture's syndrome with normal renal function.

Two male patients with Goodpasture's syndrome manifesting as severe pulmonary hemorrhage with minimal renal abnormalities are described. Both patients had microscopic hematuria with normal renal function, and one had transient proteinuria. Renal glomerular histology was normal and electron microscopic findings revealed no electron-dense deposits, but immunofluorescence of immunoglobulin G (IgG) was positive in a linear fashion along glomerular capillary basement membranes in both patients. Pulmonary hemorrhage was arrested following prednisone therapy, and both patients have normal pulmonary and renal function at five and 13 months of follow-up. The literature on patients with Goodpasture's syndrome, pulmonary hemorrhage and normal renal function with minimal proteinuria is reviewed. It is suggested that a subset of patients with Goodpasture's syndrome have pulmonary hemorrhage as their major manifestation. Since prednisone seems to have an apparent beneficial effect on pulmonary hemorrhage, and relatively good prognosis, this diagnosis should be considered in patients with idiopathic pulmonary hemorrhage.