Granulomatosis with polyangiitis natural history, complications and prognosis: Difference between revisions
No edit summary |
|||
(3 intermediate revisions by 2 users not shown) | |||
Line 1: | Line 1: | ||
__NOTOC__ | __NOTOC__ | ||
{{Wegener's granulomatosis}} | {{Wegener's granulomatosis}} | ||
{{CMG}}{{APM}}{{AE}}{{ | {{CMG}}{{APM}}{{AE}}{{ADS}}{{KW}} | ||
== Overview == | == Overview == | ||
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 of Granulomatosis with polyangiitis include, [[vision loss]], subglottic manifestations, [[hearing loss]], [[renal failure]] and increased infections with prolonged [[immunosuppressant]] therapy. | ||
== Natural History == | == 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.<ref name="pmid13560836" /> | 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">{{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 == | == Complications == | ||
Possible complications of Granulomatosis with polyangiitis include: | Possible complications of Granulomatosis with polyangiitis include: | ||
* Vision loss | * [[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]] | ||
* Infections with prolonged | * Infections with prolonged [[immunosuppressants]]<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> | ||
==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 | * Aggressive treatment with [[corticosteroids]] and [[immunosuppressants]] | ||
'''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 | ||
* Leukocytosis | * [[Leukocytosis]] | ||
* Erythrocytes in urinary sediment | * Erythrocytes in urinary sediment | ||
* 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}} | ||
[[Category:Arthritis]] | [[Category:Arthritis]] |
Latest revision as of 20:54, 2 April 2018
Granulomatosis with polyangiitis Microchapters |
Differentiating Granulomatosis with polyangiitis from other Diseases |
---|
Diagnosis |
Treatment |
Case Studies |
Granulomatosis with polyangiitis natural history, complications and prognosis On the Web |
American Roentgen Ray Society Images of Granulomatosis with polyangiitis natural history, complications and prognosis |
FDA on Granulomatosis with polyangiitis natural history, complications and prognosis |
CDC on Granulomatosis with polyangiitis natural history, complications and prognosis |
Granulomatosis with polyangiitis natural history, complications and prognosis in the news |
Blogs on Granulomatosis with polyangiitis natural history, complications and prognosis |
Directions to Hospitals Treating Granulomatosis with polyangiitis |
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.