What is Goodpasture Syndrome?

Goodpasture Syndrome
Goodpasture's syndrome in a 26-year-old woman. The patient presented with rapid progressive dyspnea over 2 weeks. Twice bronchoalveolar lavages showed hemosiderin-laden macrophages in the cytology specimens. Later she was found to have positive serum level for antiglomerular basement membrane antibody, although her renal function was not obviously compromised. The patient expired despite intensive care. Microscopically, there was diffuse alveolar hemorrhage and hemosiderosis in the bilateral lung sections at the autopsy (panel (a), ×100, and panel (b), ×400). The hemosiderosis was confirmed by diffuse positive iron staining in hemosiderin-laden macrophages in panel (c) ((c), ×400). In panel (d), glomeruli were positive for linear IgG staining on immunofluorescent section, confirming antiglomerular basement membrane disease ((d), ×400), but no crescent formation was identified in the glomeruli. Overall autopsy findings were consistent with Goodpasture's syndrome with dominant pathologic changes in her lungs. Top Differential Diagnosis Should Be Microscopic Polyangiitis in ANCA-Positive Patient with Diffuse Pulmonary Hemorrhage and Hemosiderosis. Ward ND, Cosner DE, Lamb CA, Li W, Macknis JK, Rooney MT, Zhang PL - Case reports in pathology (2014). Not Altered. CC.

Goodpasture syndrome is inflammation of the glomerulus with or without pieces of evidence of pulmonary hemorrhage in the presence of antibodies known as the anti-glomerular basement antibodies caused by the body producing antibodies to fight the lung tissues.

What is the Pathology of Goodpasture Syndrome?

The pathology of Goodpasture syndrome is: 

-Etiology: The cause of Goodpasture syndrome is corrosion and disturbance of the normal morphology of the tissue mostly caused by exposure to hydrocarbons, cocaine inhalation, smoking and lymphocyte depletion therapy.

-Genes involved: Unknown.

-Pathogenesis: The sequence of events that lead to Goodpasture syndrome occurs when there is the binding of the antibodies on the alpha 3 collagen causing pulmonary hemorrhage and glomerulonephritis.

-Histology: The histology associated with Goodpasture shows crescentic glomerulonephritis which in turn changes to fibrotic glomerulosclerosis.

How does Goodpasture Syndrome Present?

Patients with Goodpasture syndrome typically is higher in young males and older female.it occurs in two age ranges 20-30 years and 60-80 years respectively. symptoms, features, and clinical findings associated with Goodpasture syndrome include dyspnea, fatigue, cough, hematuria, and hemoptysis.

How is Goodpasture Syndrome Diagnosed?

Goodpasture syndrome is diagnosed mostly through a biopsy of the kidneys since it gives accurate results. Another lab test done is the confirmation of the cytoplasmic antineutrophilic antibodies in the bloodstream.

How is Goodpasture Syndrome Treated?

Goodpasture syndrome is treated by the use of three guiding principles they involve; plasmapheresis which is the removal of the antibodies circulating in the system, use of immunosuppressive medication which stops the production of more antibodies, and getting rid of the provoking agents.

What is the Prognosis of Goodpasture Syndrome?

The prognosis of Goodpasture syndrome is good since those who are diagnosed with, they have a 5-year survival rate once they are adhering to the treatments.

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Last updated on May 24, 2022