Goodpasture syndrome

Goodpasture's disease or anti-glomerular basement membrane disease is an autoimmune disease, belonging to the group of alveolar or pulmonary hemorrhage diseases, which usually ends in interstitial lung disease and is characterized by the production of anti-basement membrane antibodies both from the renal glomerulus (MBG) and from the pulmonary alveoli.

History

This syndrome was first recognized by Ernest William Goodpasture in 1919 in an eighteen-year-old patient who suffered acutely from hemoptysis and hematuria shortly after recovering from influenza. Since then, this type of pathology, without being very frequent, has been well typified and there have been numerous studies that have sought to clarify its pathogenesis and its most appropriate treatment.

Causes

GN is caused by specific IgG antibodies against antigens present in the GBM. The Goodpasture antigen is located in the carboxyterminal non-collagenous region (NC1) of the a3 chain of type IV collagen of the GBM. the COL4A3 gene is located in the region q35-37 of chromosome 2. Anti-GBM antibodies also react with the basal membrane of the pulmonary alveoli).

Etiopathogenesis

Crescentic Glomerulonephritis in Goodpasture Syndrome

Although there have been many advances that have allowed us to better understand this syndrome, its etiopathogenesis remains to be conclusively determined, despite the fact that various agents, such as the inhalation of volatile hydrocarbons or the influenza virus, have been closely involved.

The etiology of Goodpasture syndrome remains unknown, although it is frequently preceded by a pulmonary infection or with manifestations similar to a viral disease. It has also been involved with hydrocarbon exposure and penicillamine administration (Sternlieb, 1975).

Clinical manifestations

Goodpasture syndrome usually presents between 16-30 years of age. It is recognized that pulmonary manifestations usually precede overt renal disease.

Initial symptoms are 95% dominated by hemoptysis, dyspnea on exertion, weakness, fatigue, Hb < 12 g/dL, leukocytosis > 10,000 mm³, proteinuria, and red and white cell casts on urogram.

Pulmonary hemorrhage may precede evidence of renal damage by weeks or months or may even be the only manifestation.