What is anti-GBM disease?

Anti-GBM disease is a disorder in which your body’s immune system creates antibodies that attack your kidneys and lungs. As a result, you may develop one or both of these conditions. glomerulonephritis link—inflammation of the glomeruli, tiny units in your kidney that filter wastes and extra fluid from your blood.

What causes anti-GBM disease?

Causes. Anti-GBM disease is an autoimmune disorder. It occurs when the immune system mistakenly attacks and destroys healthy body tissue. People with this syndrome develop substances that attack a protein called collagen in the tiny air sacs in the lungs and the filtering units (glomeruli) of the kidneys.

How is anti-GBM treated?

Standard treatment for anti-GBM disease includes plasmapheresis, to rapidly remove pathogenic autoantibody, along with cyclophosphamide and corticosteroids, to inhibit further autoantibody production and to ameliorate end-organ inflammation.

Is anti-GBM curable?

In conclusion, anti-GBM antibody disease is quite a rare but treatable cause of renal failure in childhood, in which prompt diagnosis and initiation of accurate therapy can be started as early as possible.

How can you tell the difference between Wegener’s and Goodpasture’s?

The typical lesion in Goodpasture’s syndrome is hæmorrhage into the lungs giving rise eventually to pulmonary siderosis, whereas in Wegener’s syndrome there is replacement of the lining of bronchi and of accessory nasal sinuses by necrotizing granulomatous tissue which may simulate carcinoma or tuberculosis.

Can anti-GBM reoccur?

Recurrent anti-GBM antibody disease in the renal allograft is also very rare, with only four case reports since 1973.

What is the most common cause of death in Goodpasture’s syndrome?

Untreated, Goodpasture syndrome can cause inflammation of the kidneys (glomerulonephritis) and can lead to permanent kidney failure. The disorder can cause severe bleeding in the lungs, which is the main cause of death from Goodpasture syndrome.

What are the final stages of GBM?

Table 2.

Symptoms	Number of patients (%)
Drowsiness/progressive loss of consciousness	48 (87)
Dysphagia	39 (71)
Progressive focal neurological deficits (motor, dysphasia)	28 (51)
Seizures	25 (45)

Is Wegener granulomatosis an autoimmune disease?

Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is a rare multisystem autoimmune disease of unknown etiology. GPA is one of the antineutrophil cytoplasmic antibody (ANCA)–associated vasculitic disorders.

Is Wegener’s disease contagious?

It’s not contagious, and there’s no evidence that it’s inherited. The condition can lead to inflamed, narrowed blood vessels and harmful inflammatory tissue masses (granulomas).

Is anti-GBM nephritic or nephrotic?

Classic anti-glomerular basement membrane (GBM) nephritis is clinically and pathologically the most aggressive form of glomerulonephritis. Patients typically present with nephritic syndrome and rapidly progressive acute renal failure.

What are pulmonary-renal syndromes?

Pulmonary-renal syndromes or lung-kidney syndromes are clinical syndromes defined by a combination of diffuse alveolar haemorrhage (DAH) and glomerulonephritis.

How is uncategorizable pulmonary-renal syndrome (PRS) treated?

Uncategorizable pulmonary-renal syndrome, in which double ANCAs and anti-GBM antibodies are negative, is extremely rare and no effective treatment has been established. Since PRS is fatal unless treated promptly, early diagnosis and timely initiation of treatment are crucial for good patient outcome.

What is the prognosis of uncategorizable pulmonary-renal syndrome?

Conclusion Uncategorizable pulmonary-renal syndrome, in which double ANCAs and anti-GBM antibodies are negative, is extremely rare and no effective treatment has been established. Since PRS is fatal unless treated promptly, early diagnosis and timely initiation of treatment are crucial for good patient outcome.