Search:

Goodpasture's syndrome - Causes, Symptoms, and Treatment

Definition

Goodpasture's syndrome is a rare disease. It is mostly affect the lungs and kidneys. In Goodpasture's syndrome, the immune system makes antibodies that attack the lungs and kidneys. It was first described by Ernest Goodpasture in 1919, and subsequently his name was given to the disease.

Causes

Goodpasture's Syndrome is an autoimmune disorder. In this case, antibodies form against a certain type of protein called collagen. The collagen is present in the alveoli (tiny air sacs in the lungs) and in the glomeruli (the filtering units of the kidney). These antibodies are called anti-glomerular basement membrane antibodies (or anti-GBM antibodies).
The antibody attack leads to bleeding in the air sacs, which causes shortness of breath, cough, and bloody sputum. It also causes inflammation in the glomeruli of the kidney, which causes blood in urine (hematuria), protein in the urine (proteinuria), or kidney failure.

Symptoms

Symptoms can quickly become severe: Breathing can fail, and large amounts of blood can be lost. At the same time, the kidneys can rapidly fail. There may be small amounts of blood in the urine. Another common symptoms are :-
Chills and fever
Nausea and vomiting
Weight loss
Chest pain
Anemia
Massive pulmonary haemorrhage can cause respiratory failure.
There is a rapidly progressive glomerulonephritis that may lead to acute renal failure and volume overload
Arthralgia.
High blood pressure (hypertension)
Less frequent urination than usual

Signs vary over time and range from clear lungs on auscultation to crackles and rhonchi. Some patients have peripheral edema and pallor from anemia.

Treatment

The main goal is to remove the circulating antibodies from the blood. An early diagnosis is very important. The patient's outlook is much worse if the kidneys are already severely damaged when treatment begins. Hospitalization is required for diagnosis and treatment of many forms of acute nephritic syndrome. The cause must be identified and treated.
Anti-inflammatory and cytotoxic agents (such as prednisone or cyclophosphamide) may be needed. If kidney failure becomes severe, dialysis may be needed to substitute for the kidney's normal functioning. Kidney transplantation may be performed in patients who suffer irreversible loss of kidney function. The nephrologist (a specialist physician) would usually wait for the levels of circulating anti-GBM antibodies to drop before proceeding with the transplant.