Nama : Nisrina Kharida

Nama Legiun : Immunology

GOODPASTURE SYNDROME

Goodpasture syndrome is a severe illness caused by the formation of

antibodies to the glomerular basement membrane and alveolus with consequential
damage to renal and pulmonary function. With current therapy, long-term survival
is more than 50%. Before, the mortality was higher than 90%. In many cases, the
disease began as dysuria, continued as anaemic syndrome, and ended with the
development of end-stage renal failure.
Immunosuppressive therapy with pulses doses of methylprednisolone and
cyclophospamide has put the disease into remission, but the permanent impairment
of renal function remained. Early diagnosis of Goodpasture syndrome helps
preserve renal function and improves patients’ survival. In patients who achieve
remission, a kidney transplant can be considered.
Goodpature syndrome is a rare clinical entity characterized by rapidly
progressive glomerulonephritis, diffuse pulmonary hemorrhage and the presence of
sirculating autoantibodies to the glomerular basement membrane (GBM).
Autoantibodies bind to reactive epitopes of noncollagenous domain of the collagen
type IV α-3 chain in glomerular and alveolar basement membranes. Autoantibodies
activate the complement cascade resulting in tissue injury by the type II
hypersensitivity reaction according to the coombs and Gell classification of
antigen-antibody reactions. Prognostic factors include the renal excretory function
and the degree of renal and lung damage at the time of presentation. Prompt
diagnosis and early and adequate medical treatment is vital for patients. Clinical
treatment must be aggresive in order to achieving better outcome.
Symptoms may occur very slowly over months or even years, but they often
develop very quickly over days to weeks. Loss of appetite, fatigue, and weakness
are common early symptoms. Lung symptoms may include, coughing up blood, dry
cough, shortness of breath. Kidney and other symptoms include, bloody urine,
burning sensation when urinating, nausea and vomiting, pale skin, adn swelling in
any area of the body, especially in the legs.
The main goal is to remove the harmful antibodies from the blood. Treatment
may include:
 Plasmapheresis, which removes harmful antibodies to help reduce
inflamation in the kidneys and lungs.
 Corticosteroid medicines (such as prednisone) and other drugs, which
suppress or quiet the immune system.
 Medicines such as angiotensin-converting enzyme (ACE) inhibitors and
angiotensin receptor blockers (ARBs), which help control blood pressure.
 Dialysis, which may be done if kidney failure can no longer function.
In other case may be told to limit your intake of salt and fluids to control swelling
and a low-to-moderate protein diet may be recommended.
DAFTAR PUSTAKA
1. Adeel Nasrullah, Zainab Fatima, Anam Javed, Usman Tariq, Muhammad
Sabih Saleem,Nasrullah. 2019. A Case of Anti-glomerular Basement
Disease Without Pulmonary Involvement. Ncbi Publishing.
2. Stephen P. McAdoo, Charles D. Pusey. 2017. Anti-Glomerular Basement
Membrane Disease. Cjasn Publishing.
3. Greco, Antonio, et al. 2015. Goodpasture's syndrome: a clinical update.
Ncbi Publishing.
4. Devrieze, Bradley W, Hurley, John A. 2017. Goodpasture syndrome (anti-
glomerular basement membrane antibody disease). StatPearls Publishing.
5. Huart, Antoine, et al. 2016. Outcomes of patients with Goodpasture
syndrome: a nationwide cohort-based study from the French Society of
Hemapheresis. Journal of autoimmunity.