Granulomatosis with polyangiitis natural history, complications and prognosis
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2]Associate Editor(s)-in-Chief: Amandeep Singh M.D.[3]Krzysztof Wierzbicki M.D. [4]
Overview
If left untreated, Granulomatosis with polyangiitis may progress to morbidity and mortality. Complications of Granulomatosis with polyangiitis include, vision loss, subglottic manifestations, hearing loss, renal failure and increased infections with prolonged immunosuppressant therapy.
Natural History
If left untreated, Granulomatosis with polyangiitis may progress to morbidity and mortality as soon as 4 weeks, with an average estimated mortality of 5 months.[1]
Complications
Possible complications of Granulomatosis with polyangiitis include:
- Vision loss[2]
- Subglottic manifestations[3]
- Hearing loss[4]
- Renal failure
- Infections with prolonged immunosuppressants[5]
Prognosis
In the past the prognosis of Granulomatosis with polyangiitis was fatal with a 1 year survival rate of 18%. Today, the prognosis of Granulomatosis with polyangiitis has improved with the introduction of corticosteroids and cyclophosphamide.[6]
The following are favorable prognostic factors:
- Diagnosed at an earlier age
- Aggressive treatment with corticosteroids and immunosuppressants
The following are poor prognostic factors:
- Presence of crescent glomeruli
- Reduced renal function
- Leukocytosis
- Erythrocytes in urinary sediment
- Presence of proteinuria
The current survival rate of Granulomatosis with polyangiitis is estimated to be 95% in 1 year, 83% in 5 years, and 65% in 10 years.[7]
References
- ↑ WALTON EW (1958). “Giant-cell granuloma of the respiratory tract (Wegener’s granulomatosis)”. Br Med J. 2 (5091): 265–70. PMC 2026251. PMID 13560836.
- ↑ Kubaisi B, Abu Samra K, Foster CS (2016). “Granulomatosis with polyangiitis (Wegener’s disease): An updated review of ocular disease manifestations”. Intractable Rare Dis Res. 5 (2): 61–9. doi:10.5582/irdr.2016.01014. PMC 4869584. PMID 27195187.
- ↑ Laudien M (2015). “Orphan diseases of the nose and paranasal sinuses: Pathogenesis – clinic – therapy”. GMS Curr Top Otorhinolaryngol Head Neck Surg. 14 ( ): Doc04. doi:10.3205/cto000119. PMC 4702053. PMID 26770278.
- ↑ Bavelloni A, Piazzi M, Raffini M, Faenza I, Blalock WL (2015). “Prohibitin 2: At a communications crossroads”. IUBMB Life. 67 (4): 239–54. doi:10.1002/iub.1366. PMID 25904163.
- ↑ Masiak A, Struk-Panfill M, Zdrojewski Z (2015). “Infectious complication or exacerbation of granulomatosis with polyangiitis?”. Reumatologia. 53 (5): 286–91. doi:10.5114/reum.2015.55833. PMC 4847319. PMID 27407261.
- ↑ Pierrot-Deseilligny Despujol C, Pouchot J, Pagnoux C, Coste J, Guillevin L (2010). “Predictors at diagnosis of a first Wegener’s granulomatosis relapse after obtaining complete remission”. Rheumatology (Oxford). 49 (11): 2181–90. doi:10.1093/rheumatology/keq244. PMID 20675708.
- ↑ Mohammad AJ, Jacobsson LT, Westman KW, Sturfelt G, Segelmark M (2009). “Incidence and survival rates in Wegener’s granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and polyarteritis nodosa”. Rheumatology (Oxford). 48 (12): 1560–5. doi:10.1093/rheumatology/kep304. PMID 19797309.
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