HSC Section 6 Nov2016 Green Book

Reprinted by permission of JAMA Otolaryngol Head Neck Surg. 2013; 139(1):76-81.

ORIGINAL ARTICLE

Clinical Manifestations and Treatment of Idiopathic and Wegener Granulomatosis–Associated Subglottic Stenosis

Stanford C. Taylor, BS; Daniel R. Clayburgh, MD, PhD; James T. Rosenbaum, MD; Joshua S. Schindler, MD

Objective : To compare and contrast the manifesta- tions and surgical management of subglottic stenosis in patients with airway obstruction attributed to granulo- matosis with polyangiitis (GPA), previously known as Wegener granulomatosis, and those with idiopathic sub- glottic stenosis (iSGS). Design : Retrospective medical chart review. Review of subglottic stenosis cases seen in the otolaryngology de- partment of an academic medical center from 2005 through 2010. Data were obtained on disease presenta- tion, operative management. and findings. Participants : A total of 24 patients with iSGS and 15 patients with GPA-associated subglottic stenosis (GPA- SGS). Results : All individuals with iSGS were female, and 40% of patients with GPA-SGS were male ( P .01). Patients Setting : Tertiary referral center.

with iSGS tended to have a higher Myer-Cotton stenosis grade at the time of dilation than those with GPA-SGS ( P =.02). Individuals with GPA-SGS were more likely to undergo tracheotomy as a result of disease-related com- plications than individuals with iSGS ( P .01). No pa- tients with an open airway reconstruction in the iSGS group required follow-up mechanical dilation. In con- trast, all patients with open airway reconstructions in the GPA-SGS group underwent more than 1 subsequent air- way dilation ( P .01). Conclusions : While surgical utilization is the mainstay of treatment in iSGS and GPA-SGS, iSGS occurs almost exclusively in females and presents with a greater de- gree of stenosis at the time of endoscopic dilation. In con- trast, GPA-SGS is associated with greater rates of trache- otomy. Open airway reconstruction may be used in the treatment of iSGS and GPA-SGS but is much more ef- fective in iSGS.

JAMA Otolaryngol Head Neck Surg. 2013;139(1):76-81

A CQUIRED SUBGLOTTIC STE - nosis (SGS) describes air- way narrowing in the area of the cricoid and is asso- ciated with prolonged in- tubation or external trauma. Other pa- tients acquire SGS from a systemic autoimmune etiology, such as granulo- matosis with polyangiitis (GPA), previ- ously known as Wegener granulomato- sis. However, in about 20% of cases there is no identifiable precipitant, and pa- tients are therefore classified as having id- iopathic subglottic stenosis (iSGS). 1 While posttraumatic SGS is often evident based on patient history, discriminating be- tween iSGS and GPA-SGS can be diagnos- tically challenging, especially in cases in which SGS is the presenting symptom of GPA. Presenting symptoms of dyspnea, noisy breathing, and voice changes can oc- cur in both groups. 2,3 Anatomically, the co- horts appear different, as scar formation

in patients with GPA-SGS and iSGS typi- cally involves soft tissue (membranous ste- nosis), but intubation and trauma- related SGS typically leads to firm, cartilaginous scar tissue. 4 GPA-SGS is di- agnosed based on the presence of autoan- tibodies or characteristic findings on bi- opsy, but negative results cannot reliably rule out autoimmune-mediated SGS, es- pecially when the disease has limited or- gan involvement. 5,6 Indeed, given themany similarities between patients with iSGS and those with GPA-SGS, some have sug- gested that iSGS may represent a spec- trum of autoimmune-mediated SGS that is not yet fully understood and that GPA- SGS is but a part of this autoimmune SGS spectrum. Despite the similarities noted between iSGS and GPA-SGS, there is a paucity of data in the literature directly comparing these entities, specifically with regard to therapeutic and surgical outcomes. Given

Author Af Medicine ( Departme Otolaryng Surgery (D Schindler) Arthritis a Diseases, Medicine ( Oregon He University,

Author Affiliations: School of Medicine (Mr Taylor), Department of Otolaryngology–Head and Neck Surgery (Drs Clayburgh and Schindler), and Division of Arthritis and Rheumatic Diseases, Department of Medicine (Dr Rosenbaum), Oregon Health and Science University, Portland.

JAMA OTOLARYNGOL HEAD NECK SURG/VOL 139 (NO. 1), JAN 2013 WWW.JAMAOTO.COM

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