Granulomatosis with polyangiitis
| Granulomatosis with polyangiitis | |
|---|---|
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| Micrograph showing features characteristic of granulomatosis with polyangiitis - a vasculitis and granulomas with multi-nucleated giant cells. H&E stain. | |
| Classification and external resources | |
| Specialty | rheumatology |
| ICD-10 | M31.3 |
| ICD-9-CM | 446.4 |
| DiseasesDB | 14057 |
| MedlinePlus | 000135 |
| eMedicine | med/2401 |
| Patient UK | Granulomatosis with polyangiitis |
| MeSH | D014890 |
Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG), is a systemic disorder that involves both granulomatosis and . It is a form of vasculitis (inflammation of blood vessels) that affects small- and medium-size vessels in many organs. Damage to the lungs and kidneys can be fatal. It requires long-term immunosuppression. The condition was originally named for Friedrich Wegener, who described the disease in 1936. As a response to Wegener's association with the German Nazi party, professional bodies and journals have replaced his name with a descriptive name. However, the older name is still often seen.
Granulomatosis with polyangiitis is part of a larger group of vasculitic syndromes called systemic vasculitides or necrotizing vasculopathies, all of which feature an autoimmune attack by an abnormal type of circulating antibody termed ANCAs (antineutrophil cytoplasmic antibodies) against small and medium-size blood vessels. Apart from GPA, this category includes eosinophilic granulomatosis with polyangiitis (EGPA) and microscopic polyangiitis. Although GPA affects small- and medium-size vessels, it is formally classified as one of the small vessel vasculitides in the Chapel Hill system.
Initial signs are extremely variable, and diagnosis can be severely delayed due to the nonspecific nature of the symptoms. In general, rhinitis is the first sign in most people.
Its causes are unknown, although microbes, such as bacteria and viruses, as well as genetics have been implicated in its pathogenesis.
Inflammation with granuloma formation against a nonspecific inflammatory background is the classical tissue abnormality in all organs affected by GPA.
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