User:Mr. Ibrahem/Goodpasture syndrome
| Goodpasture syndrome | |
|---|---|
| Other names | Goodpasture’s disease, antiglomerular basement antibody disease, anti-GBM disease |
 | |
| Micrograph of a crescentic glomerulonephritis due to GPS, PAS stain | |
| Specialty | Nephrology |
| Symptoms | blood in the urine, protein in the urine, coughing up blood[1] |
| Complications | Acute kidney failure[1] |
| Usual onset | ~25 years old[2] |
| Causes | Environmental trigger in those who are genetically susceptible[1] |
| Risk factors | HLA-DR15[1] |
| Diagnostic method | Blood tests, kidney biopsy[1] |
| Differential diagnosis | Granulomatosis with polyangiitis, lupus, microscopic polyangiitis, IgA nephropathy, acute glomerulonephritis[1] |
| Treatment | Corticosteroids, cyclophosphamide, plasmapheresis, hemodialysis, mechanical ventilation[1] |
| Prognosis | 5-year survival rate > 80% |
| Frequency | Rare[1] |
Goodpasture syndrome (GPS), also known as anti-glomerular basement membrane disease, is a disease that involves the kidneys and lungs.[1] Symptom may include blood in the urine, protein in the urine, and coughing up blood.[1] Shortness of breath, tiredness, and chest pain may also occur.[2] Acute kidney failure may result.[1]
It is believed to occur following an environmental trigger in people who are genetically susceptible.[1] Risk factors include HLA-DR15.[1] Triggers may include certain medications, cocaine, smoking, and infection by influenza.[1] The underlying mechanism involves autoantibodies against the basement membrane.[1] The diagnosis may be confirmed by blood testing looking for specific antibodies or a kidney biopsy.[1]
Treatment is generally with medications that suppress the immune system such as corticosteroids or cyclophosphamide.[1] Plasmapheresis may be done daily to remove antibodies from the blood.[1] Other required efforts may include hemodialysis or mechanical ventilation.[1] With treatment the 5-year survival rate is greater than 80% and less than 30% require long term dialysis.[1]
Goodpasture syndrome is a rare, affecting about one in a million people per year.[1] Males are more commonly affected than females.[2] Those around the age of 25 are most commonly affected.[2] It is named after the American pathologist Ernest Goodpasture who first described the condition in 1919.[1][3][4]
References[edit]
- ^ a b c d e f g h i j k l m n o p q r s t u v DeVrieze, BW; Hurley, JA (January 2020). "Goodpasture Syndrome". PMID 29083697.
{{cite journal}}: Cite journal requires|journal=(help) - ^ a b c d "Goodpasture Syndrome". NORD (National Organization for Rare Disorders). Retrieved 20 January 2021.
- ^ Goodpasture EW (1919). "The significance of certain pulmonary lesions in relation to the etiology of influenza". Am J Med Sci. 158 (6): 863–870. doi:10.1097/00000441-191911000-00012.
- ^ Salama AD, Levy JB, Lightstone L, Pusey CD (September 2001). "Goodpasture's disease". Lancet. 358 (9285): 917–920. doi:10.1016/S0140-6736(01)06077-9. PMID 11567730.