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Granulomatosis with polyangiitis (Wegener’s) (GPA)

What is granulomatosis with polyangiitis (Wegener's) (GPA)?

Granulomatosis with polyangiitis (Wegener's) (GPA) is a rare form of vasculitis mainly involving small and medium-sized blood vessels. The disease can affect most parts of the body. It commonly affects the sinuses, nose, throat, lungs, ears, eyes, kidneys, skin, joints, brain and other parts of the nervous system. Not everyone with GPA is affected in the same way. Some patients have mild disease, while others may have severe damage to these organs which can be life-threatening.

What is “granulomatosis with polyangiitis”?

Granulomatosis with polyangiitis (GPA) is a new term introduced to replace the name Wegener’s granulomatosis. As the term is being adopted, the terminology “granulomatosis with polyangiitis (Wegener’s)” has been proposed.

Who gets granulomatosis with polyangiitis?

Granulomatosis with polyangiitis (Wegener's) (GPA) occurs in both men and women and can affect children and adults. Although Caucasians are affected more often, people all over the world can get GPA.

What causes granulomatosis with polyangiitis?

Granulomatosis with polyangiitis (Wegener's) (GPA) is thought to be an auto-immune disease for which there is no known cause.

How is granulomatosis with polyangiitis diagnosed?

The diagnosis of GPA is made by combining clinical features with laboratory tests (including tests for ANCA) and biopsy of affected tissues.

What is the treatment for granulomatosis with polyangiitis?

Treatment of GPA usually includes a combination of glucocorticoids and an immunosuppressive drug such as cyclophosphamide, methotrexate, or azathioprine. If diagnosed promptly, treatment can bring about early remission and prevent organ failure. It is a chronic disease, and although remission of symptoms is usually achieved, the relapse rate remains high.

What are the chances that my newly diagnosed vasculitis will relapse?

Reported relapse rates in granulomatosis with polyangiitis (Wegener’s) have ranged from 50-70%.

What is the difference between limited and severe granulomatosis with polyangiitis (Wegener’s)?

Not all patients with granulomatosis with polyangiitis (GPA) have similar disease. The term “limited” GPA was introduced in 1966 and was originally defined as GPA that did not involve the kidney. Unfortunately, this term has often been misinterpreted. “Limited” GPA is not equivalent to non-severe GPA as there are people who meet the definition of “limited” disease who may have very severe disease and people who do not meet the definition of limited GPA whose disease may be quite mild. Because of this, the term “limited” GPA is best avoided. It is far more helpful to view this disease in terms of the location of organ involvement and the degree of severity of the disease in each of these locations.

What is the difference between ANCA and ANA?

Both are blood tests used by doctors to help in the diagnosis of autoimmune disease. Antineutrophil cytoplasmic antibody (ANCA) is blood test commonly elevated in patients with diseases such granulomatosis with polyangiitis, microscopic polyangiitis, and Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss). Antinuclear antibody (ANA) is a blood test most often elevated in patients with systemic lupus erythematosus (“lupus”), Sjogren syndrome, scleroderma, and other types of autoimmune diseases.

Can you still be diagnosed with granulomatosis with polyangiitis (GPA, Wegener’s) if the ANCA is negative? Yes. Although ANCA is elevated in a majority of patients with granulomatosis with polyangiitis (Wegener’s) it is not detectable in all patients and is not necessary to make the diagnosis.

In ANCA-associated vasculitis, including granulomatosis with polyangiitis (Wegener’s, GPA), do increases in the ANCA tests predict disease relapse?

A general and over-simplified answer is: no. This answer is based on the statistical analysis of large groups of patients followed prospectively in clinical trials. The results from these studies have led to the general opinion held by vasculitis experts that treatment decisions should not be made based on changes in ANCA levels alone.

There are some patients, however, for whom ANCA levels track the disease activity very well. In these patients, ANCA increases do predict relapses. To know how ANCA levels relate to disease activity in individual patients, their ANCA levels need to be monitored over time.

It should also be noted that there are different ways of measuring ANCA levels. To follow serial ANCA levels they need to be measured by the same method to be able to make comparisons.

What are anti-GBM antibodies and what do they have to do with my vasculitis?

Anti-GBM antibodies are antibodies directed against glomerular basement membranes. Patients with anti-GBM disease (Goodpasture’s syndrome) will present with involvement of lungs and kidneys, so-called pulmonary-renal syndrome. This can include bleeding in the lungs (hemoptysis) that can lead to respiratory failure and bleeding in the kidney (hematuria) that can lead to rapidly progressive loss of kidney function. Goodpasture syndrome has to be considered in patients who are thought to have ANCA-related vasculitidies (granulomatosis with polyangiitis and microscopic polyangiitis) as those diseases can also present with pulmonary-renal syndrome. Measuring anti-GBM antibodies and ANCA in these patients will usually help distinguish the two conditions although there are rare cases of patients with both ANCA and anti-GBM antibodies.

What are the chances of recovering lost hearing in a patient with granulomatosis with polyangiitis (GPA, Wegener’s)?

Hearing can be transiently decreased in patients with GPA with acute otitis, because of the presence of liquid in the inner ear(s), which will regress under appropriate treatment. However, when the inner ear damage is severe, because of prolonged or multiple recurrences of otitis, and/or when the auditory nerve(s) are involved by inflammation or compression, the hearing loss can be permanent.

Can a saddle nose deformity be repaired?

Yes, a saddle nose deformity can be repaired as long as the underlying vasculitis is not active. This would require consultation with a surgeon, often times an ear nose and throat (ENT) specialist who could discuss this option with you.