Granulomatosis with polyangiitis natural history, complications and prognosis: Difference between revisions
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== Natural History == | == Natural History == | ||
If left untreated, Granulomatosis with polyangiitis may progress to morbidity and mortality. | If left untreated, Granulomatosis with polyangiitis may progress to morbidity and mortality. | ||
== Complications == | |||
==Prognosis== | ==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.<ref name="pmid20675708">{{cite journal| author=Pierrot-Deseilligny Despujol C, Pouchot J, Pagnoux C, Coste J, Guillevin L| title=Predictors at diagnosis of a first Wegener's granulomatosis relapse after obtaining complete remission. | journal=Rheumatology (Oxford) | year= 2010 | volume= 49 | issue= 11 | pages= 2181-90 | pmid=20675708 | doi=10.1093/rheumatology/keq244 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20675708 }}</ref> | |||
; | |||
The following are favorable prognostic factors: | |||
* Diagnosed at an earlier age | |||
* Administration of medications at an earlier stage | |||
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.<ref name="pmid19797309">{{cite journal| author=Mohammad AJ, Jacobsson LT, Westman KW, Sturfelt G, Segelmark M| title=Incidence and survival rates in Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and polyarteritis nodosa. | journal=Rheumatology (Oxford) | year= 2009 | volume= 48 | issue= 12 | pages= 1560-5 | pmid=19797309 | doi=10.1093/rheumatology/kep304 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19797309 }}</ref> | |||
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. | ||
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. | 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. | ||
Revision as of 15:33, 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.
Complications
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.[4]
The following are favorable prognostic factors:
- Diagnosed at an earlier age
- Administration of medications at an earlier stage
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.[5]
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.
- ↑ 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.