| Granulomatosis with polyangiitis | |
|---|---|
| Other names | Wegener's granulomatosis (WG) |
 | |
| Micrograph showing features of granulomatosis with polyangiitis – vasculitis and granulomas with multi-nucleated giant cells. H&E stain. | |
| Specialty | Rheumatology |
| Symptoms | Stuffy nose, nosebleeds, inflammation within the eye, fever, coughing up blood, leg swelling, weight loss[1][2] |
| Complications | Hearing loss, blindness, infections, kidney failure[3] |
| Usual onset | 40 to 60 yrs old[2] |
| Causes | Unknown[3] |
| Differential diagnosis | Microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, polyarteritis nodosa[2] |
| Treatment | Immunosuppressive medications (rituximab, cyclophosphamide, corticosteroids)[4] |
| Prognosis | 90% have significant problems despite treatment[4] |
| Frequency | Rare[3] |
Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG), is a long-term disorder that involves the formation of granulomas and inflammation of blood vessels (vasculitis).[3][4] It affects small- and medium-size blood vessels, mostly commonly in the upper respiratory tract, lungs, and kidneys.[3] Typical symptoms include a stuffy nose, nosebleeds, and inflammation within the eye.[1] Other symptoms may include fever, coughing up blood, leg swelling, and weight loss.[2] Onset of symptoms may be gradual or rapid.[2] Complications may include hearing loss, blindness, infections, and kidney failure.[3]
The exact cause is unclear, though it is believed to be related to a combination of genetics and environmental exposures.[3][5] It; however, is not inherited from a person's parents.[2] It is a type of autoimmune disorder, specifically a ANCA-associated vasculitis, along with microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis.[3][2] The diagnosis may be suspected based on symptoms and laboratory tests and confirmed by tissue biopsy.[2]
Treatment depends on the severity of the disease.[4][6] Severe disease is typically treated with a combination of immunosuppressive medications such as rituximab or cyclophosphamide and high-dose corticosteroids, to bring about remission of symptoms.[4] To keep the disease under control azathioprine, methotrexate, or rituximab may be used.[4][7] Plasma exchange may be used in cases with kidney injury.[8] Without treatment survival is poor; however, with treatment most live more than 8 years.[3]
The number of new cases a year is estimated at 2–14 per million people in Europe.[1] In the United States about 3 per 100,000 people are affect.[9] Onset is most often between the age of 40 and 60.[2] Males and females are affected with similar frequency.[2] GPA is rare in Japanese and African-American populations.[10] While the condition was first described in 1931 by Heinz Klinger, it was not identified as a separate condition until 1936 by Friedrich Wegener.[3]
References edit
- ^ a b c Yates, M; Watts, R (February 2017). "ANCA-associated vasculitis". Clinical Medicine (Review). 17 (1): 60–64. doi:10.7861/clinmedicine.17-1-60. PMC 6297586. PMID 28148583.
- ^ a b c d e f g h i j "Granulomatosis with Polyangiitis". NORD (National Organization for Rare Disorders). Retrieved 11 April 2021.
- ^ a b c d e f g h i j Garlapati, P; Qurie, A (January 2021). "Granulomatosis with Polyangiitis". PMID 32491759.
{{cite journal}}: Cite journal requires|journal=(help) - ^ a b c d e f "Granulomatosis with Polyangiitis (GPA) - Musculoskeletal and Connective Tissue Disorders". MSD Manual Professional Edition. Retrieved 11 April 2021.
- ^ "Granulomatosis with polyangiitis | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Retrieved 11 April 2021.
- ^ Lally, L; Spiera, R (2015). "Current therapies for ANCA-associated vasculitis". Annual Review of Medicine (Review). 66: 227–40. doi:10.1146/annurev-med-011514-023051. PMID 25341007.
- ^ Singer, O; McCune, WJ (May 2017). "Update on maintenance therapy for granulomatosis with polyangiitis and microscopic polyangiitis". Current Opinion in Rheumatology (Review). 29 (3): 248–53. doi:10.1097/BOR.0000000000000382. PMID 28306595. S2CID 35805200.
- ^ Walters, GD; Willis, NS; Cooper, TE; Craig, JC (13 January 2020). "Interventions for renal vasculitis in adults". The Cochrane database of systematic reviews. 1: CD003232. doi:10.1002/14651858.CD003232.pub4. PMID 31927782.
- ^ Pakalniskis, MG; Berg, AD; Policeni, BA; Gentry, LR; Sato, Y; Moritani, T; Smoker, WR (December 2015). "The Many Faces of Granulomatosis With Polyangiitis: A Review of the Head and Neck Imaging Manifestations". AJR. American Journal of Roentgenology (Review). 205 (6): W619–29. doi:10.2214/AJR.14.13864. PMID 26587951.
- ^ Millet, A; Pederzoli-Ribeil, M; Guillevin, L; Witko-Sarsat, V; Mouthon, L (August 2013). "Antineutrophil cytoplasmic antibody-associated vasculitides: is it time to split up the group?". Annals of the Rheumatic Diseases (Review). 72 (8): 1273–9. doi:10.1136/annrheumdis-2013-203255. PMID 23606701. S2CID 206849855.