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: Krzysztof Wierzbicki M.D. [3]Cafer Zorkun, M.D., Ph.D. [4]
Overview
If left untreated, Granulomatosis with polyangiitis may progress to morbidity and mortality. [1] Complications of Granulomatosis with polyangiitis include, vision loss[2], subglottic manifestations[3], hearing loss[4], renal failure and increased infections with prolonged immunosuppressant therapy.[5]
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
- subglottic manifestations
- hearing loss
- renal failure
- Infections with prolonged immunosuppressant's
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 immunosuppressant's
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
- ↑ 1.0 1.1 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.