Hu LH, Leitón CA, Salas MC

Language: Spanish
References: 10
Page: 1-10
PDF size: 686.95 Kb.

ABSTRACT

Associated vasculitis to antineutrophil cytoplasmatic antibodies, clasified as small vessel vasculitis that includes: polyangiitis whit granulomatosis (Wegener disease) microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis (Churg Strauss). Churg Strauss disease is one of the rarest systemic necrotizing vasculitis, but potenctially deadly. International incidieses for this disease is 2.5 cases per every 100.000 adults per year, the range of survival to 1 year is 90% and to 5 years is 62%. The Churg Strauss syndrome has a complex fisiopathology, in which genetic factors and external agentes are involved: also different types of cells, like T cells, eosinophils, B cells, and inflammatory cells. There are 3 phases on the evolution of the disease. The first phase is characterized for atopic demostrations like asthma, allergic rhinitis and nasal polyps. The second phase is characterized for periferic eosinophilia and eosinophilic infiltrates in lungs and gastrointestinal tract. During the third phase of the disease there are vasculitic demonstrations. Besides constitutional symptons there is also musculokeletal demonstrational like myalgia, arthralgia and arthritis. The Churg Strauss syndrome prognosis has improve drastically since the introduccion of corticosteroids therapy and it’s the first line of therapy. Even do the cornerstone of treatment are the corticosteroids, the severe presentation of this disease is treated in similar way as any other vasculitis associated with ANCA, whit other inmunossuppresive drugs like cyclophosphamide, however treatment is still a challenge, with limited clinical studies.

REFERENCES

1. Groh M Pagnoux C Guillevin L. Eosinop-hilic granulomatosis with polyangiitis (formerly Churg–Strauss syndrome): where are we now?. Eur Respir J. 2015; 46: 1255–1258

2. Farid-Moayer M. Churg-Strauss Syn-drome. Medscape. 2015; http://emedi-cine.medscape.com/article/333492-over-view.

3. Sánchez A González M. Poliangeítis gra-nulomatosa con eosinofilia. Revisión y re-porte de caso de una paciente con eo-sinofilia, mononeuropatía de miembro in-ferior, rinosinusitis y asma. Revista Co-lombiana de Reumatología. 2015; 22(3): 180-185.

4. Staley R. Churg-Strauss Disease Clinical Presentation. Medscape. 2104; http://emedicine.medscape.com/arti-cle/1178795-clinical

5. Vaglio A Moosig F Zwerina J. Churg-Strauss syndrome: update on pathop-hysiology and treatment. Curr Opin Rhe-umatol. 2012 Jan; 24(1): 24-30

6. Restrepo M González L Vásquez G Lon-doño D Correa L Ramírez L. Síndrome de Churg-Strauss: a propósito de un caso con manifestaciones poco usuales. Re-vista Colombiana de Reumatología. 2009; 16 (1): 112-119.

7. Comarmond C Pagnoux C Khellaf M et al. Eosinophilic granulomatosis with polyan-giitis (Churg-Strauss): clinical characte-ristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis Rheum. 2013; 65(1): 270-281.

8. Buzio C Oliva E. Diagnosis of Churg Strauss syndrome: eotaxin-3 makes it easier. Rheumatology 2011; 50: 1737-1738.

9. Terán L Ledesma Y Krengel S Lezcano D. Eotaxinas en asma bronquial y poliposis nasal. Gac Méd Méx. 2006; 142 (2): 139-144

10. Keogh K. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss sín-drome). BMJ Best Practice. 2016; http://bestpractice.bmj.com/best-prac-tice/mybp/monograph-pdf/942.pdf