AUTOIMMUNE DISORDERS OF THE LARYNX (AND OTHER STRANGE THINGS )
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1 AUTOIMMUNE DISORDERS OF THE LARYNX (AND OTHER STRANGE THINGS ) Lyndsay Madden, DO Otolaryngology/Facial Plastic Surgery PGY 5 Grandview Medical Center/OU-HCOM Dayton, Ohio
2 Rheumatoid arthritis Granulomatosis with Polyangiitis (GPA) Relapsing Polychondritis Sarcoidosis Amyloidosis Granular Cell Tumor Neurofibroma Chondrosarcoma
3 Rheumatoid Arthritis 3F : 1M, 3:1000 Larynx: 25% Symptoms: hoarseness, dysphagia, globus, stridor, throat/anterior neck pain
4 RA Active: red, swollen arytenoids Chronic: CA joint ankylosis, obliteration of joint, cricoid necrosis (late) B/L immobility (RA is destructive ) Other: Myositis, neuropathy of RLN d/t vasculitis associated with RA and postcricoid granulomas
5 Rheumatoid Arthritis Submucosal RA nodules Hoarseness Transverse white-yellow band lesions on superior surface of vocal folds Bilateral, usually not opposing
6 Bamboo Nodules
7 RA treatment Goals reduce pain, inflammation, and inability DMARDS, Corticosteroids, MTX, immunomodulators (monoclonal ab tx) Injection of steroids
8 Granulomatosis with Polyangiitis (GPA) (The Disease formerly know as Wegener s Granulomatosis) Necrotizing granulomatosis and necrotizing vasculitis ENT (larynx-sg/trachea), lungs, kidneys 10-20% WG present with SGS Dx: 80% have + C-ANCA May need tissue biopsy S/S: hoarse, cough, hemoptysis, dyspnea, stridor, wheezing
9 GPA Hallmark Path: Vasculitis Granulomatous inflammation Necrosis
10
11 GPA Tx: systemic First line: Steroids + cyclophosphamide Remission: methotrexate or azathioprine Airway systemic symptoms Dilation (balloon, CO 2 ) + intralesional steroids +/- MMC Stents Trach Tracheal resection: mature stenosis, inactive dz
12 Relapsing Polychondritis Episodic inflammation/fibrosis destroys cartilage Classic ENT manifestations of RP: Auricular, nasal, and laryngotracheal 50% have laryngotracheal disease Symptoms: hoarseness, cough, dyspnea, or pain Can develop airway obstruction Treat: medical (steroids, NSAIDS, MTX) Sometimes surgical for airway
13 RP Imaging, Histo Airway fluoroscopy: eval collapse during exhalation CT/MRI: complimentary Chondritis w/ mixed inflam cell infiltrate
14 Radiographic Imaging
15 RP - Treatment Stents Trach Acute: high-dose steroids injection? Maintenance: methotrexate, low-dose steroids Refractory: azathioprine, cyclophosphamide, cyclosporine, dapsone, or TNF alpha blockers -infliximab
16 Sarcoidosis Systemic inflammatory disease of unknown etiology Non-caseating granulomas Supraglottic >>>> subglottic involvement Thickened epiglottis - Turban like Need biopsy Treat underlying disease Surgical: dilation, steroid injections
17 Sarcoidosis Etiology: unknown Path: noncaseating granulomas 9% w/ent, 1-5% laryngeal Symptoms: mild despite extensive tissue involvement Hoarseness, dyspnea, stridor, dysphagia, cough Progression: slow, relapses/remissions
18 Amyloidosis Idiopathic Abnormal protein deposition ~300 cases of upper airway amyloidosis reported Laryngeal disease: 40 s-60 s, M>F Usually localized; rarely systemic
19 Amyloidosis Location: TVF FVF Ventricle
20 Amyloidosis Sx s: hoarseness, occ airway obstruction Requires biopsy: Congo red staining shows bright apple green birefringence under polarized light microscopy Treatment=surgical excision
21 Granular cell tumor Origin: schwann cells MC tongue/2 nd MC Larynx Associated with: overlying pseudoepitheliomatous hyperplasia of mucosa Location: middle-posterior TVF CC: hoarseness Tx: complete local excision
22 Neurofibroma Solitary > w/ von Recklinghausen s dz Symptoms: hoareseness, dyspnea, dysphagia Location: arytenoid, AEF Size: 2-8 cm! (19 cases) Tx: conservative (since benign) Endoscopic Lateral pharyngotomy, laryngofissure, lateral thyrotomy
23 Pemphigus Loosening epidermal-dermal jxn blebs But, usu don t see blebs sloughed when swallow Tan fibrinous base w/halo of erythema Acantholyic subepithelial lesions w/ BM involvement Direct immunofluorescence = intracellular pattern Laryngeal not uncommon if nasal complaints Symptoms: hoarseness, progressive stridor, dyspnea Location: MC: oral cavity Supraglottis laryngeal surface epiglottis, AEF, arytenoid Tx: oral steroids (high-dose) taper to maintenance
24 Chondrosarcoma Chondrosarcoma common in bone but rare in larynx (<1% of larynx cancers) 75% arise from the cricoid (usually posterior lamina) 6 th or 7 th decade Presents as stridor or dyspnea (occ dysphonia) Surgical excision
25
26 THANK YOU!!! Thanks to Dr. Libby Smith Questions???
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