Granulomatosis with polyangiitis natural history, complications and prognosis: Difference between revisions
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==Overview== | ==Overview== | ||
If left untreated, Granulomatosis with polyangiitis may progress to morbidity and mortality. <ref name="pmid13560836">{{cite journal| author=WALTON EW| title=Giant-cell granuloma of the respiratory tract (Wegener's granulomatosis). | journal=Br Med J | year= 1958 | volume= 2 | issue= 5091 | pages= 265-70 | pmid=13560836 | doi= | pmc=2026251 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=13560836 }}</ref> Complications of Granulomatosis with polyangiitis include, perforation of the globe, peripheral ulcerative keratitis<ref name="pmid27195187">{{cite journal| author=Kubaisi B, Abu Samra K, Foster CS| title=Granulomatosis with polyangiitis (Wegener's disease): An updated review of ocular disease manifestations. | journal=Intractable Rare Dis Res | year= 2016 | volume= 5 | issue= 2 | pages= 61-9 | pmid=27195187 | doi=10.5582/irdr.2016.01014 | pmc=4869584 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27195187 }}</ref> and increased infections with prolonged immunosuppressant therapy.<ref name="pmid27407261">{{cite journal| author=Masiak A, Struk-Panfill M, Zdrojewski Z| title=Infectious complication or exacerbation of granulomatosis with polyangiitis? | journal=Reumatologia | year= 2015 | volume= 53 | issue= 5 | pages= 286-91 | pmid=27407261 | doi=10.5114/reum.2015.55833 | pmc=4847319 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27407261 }}</ref> | |||
== Natural History == | |||
If left untreated, Granulomatosis with polyangiitis may progress to morbidity and mortality. | |||
==Prognosis== | ==Prognosis== | ||
25 to 40% of patients suffer from flare-ups, but a majority responds well to treatment. Anatomical problems ([[sinusitis]], tracheal stenosis) may require surgery in a small proportion. Relapses can be long and troublesome. | 25 to 40% of patients suffer from flare-ups, but a majority responds well to treatment. Anatomical problems ([[sinusitis]], tracheal stenosis) may require surgery in a small proportion. Relapses can be long and troublesome. | ||
Revision as of 14:53, 9 November 2016
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, perforation of the globe, peripheral ulcerative keratitis[2] and increased infections with prolonged immunosuppressant therapy.[3]
Natural History
If left untreated, Granulomatosis with polyangiitis may progress to morbidity and mortality.
Prognosis
25 to 40% of patients suffer from flare-ups, but a majority responds well to treatment. Anatomical problems (sinusitis, tracheal stenosis) may require surgery in a small proportion. Relapses can be long and troublesome. With the appropriate treatment, the outlook is good for people with Wegener's granulomatosis. In a study of 158 patients who were treated with prednisone and cyclophosphamide at the National Institutes of Health (NIH), 91 percent of them markedly improved. After 6 months to 24 years of follow-up, 80 percent of the patients survived.
Approximately half of people with Wegener's granulomatosis may experience a return of their disease. This occurs most frequently within 2 years of stopping medicine but can occur at any point both during treatment or after stopping treatment. Thus, it is extremely important that people continue to see their doctors regularly, both while they are on these medicines, as well as after the medicines have been stopped.
Complications
Long-term complications are very common (86%): mainly chronic renal failure, hearing loss and deafness.
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.
- ↑ 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.