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, | 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, vision loss<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>, subglottic manifestations<ref name="pmid26770278">{{cite journal| author=Laudien M| title=Orphan diseases of the nose and paranasal sinuses: Pathogenesis - clinic - therapy. | journal=GMS Curr Top Otorhinolaryngol Head Neck Surg | year= 2015 | volume= 14 | issue= | pages= Doc04 | pmid=26770278 | doi=10.3205/cto000119 | pmc=4702053 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26770278 }}</ref>, hearing loss<ref name="pmid25904163">{{cite journal| author=Bavelloni A, Piazzi M, Raffini M, Faenza I, Blalock WL| title=Prohibitin 2: At a communications crossroads. | journal=IUBMB Life | year= 2015 | volume= 67 | issue= 4 | pages= 239-54 | pmid=25904163 | doi=10.1002/iub.1366 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25904163 }}</ref>, renal failure 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 == | == 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 as soon as 4 weeks, with an average estimated mortality of 5 months.<ref name="pmid13560836" /> | ||
== Complications == | == Complications == | ||
Possible complications of Granulomatosis with polyangiitis include: | |||
* Vision loss | |||
* subglottic manifestations | |||
* hearing loss | |||
* renal failure | |||
* Infections with prolonged immunosuppressant's | |||
==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> | 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: | '''The following are favorable prognostic factors:''' | ||
* Diagnosed at an earlier age | * Diagnosed at an earlier age | ||
* | * Aggressive treatment with corticosteroids and immunosuppressant's | ||
'''The following are poor prognostic factors:''' | |||
The following are poor prognostic factors: | |||
* Presence of crescent glomeruli | * Presence of crescent glomeruli | ||
* Reduced renal function | * Reduced renal function | ||
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* Presence of proteinuria | * 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> | 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> | ||
==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} |
Revision as of 16:00, 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, 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.