Sarcoidosis is a multi-system disorder with lung as the prime target organ. Laryngeal involvement is seen in only 0.5% to 8.3% of patients and if unrecognized and untreated may progress to upper airway obstruction. Hoarseness is the usual early symptom. We describe a patient with sarcoidosis of 13 years duration, who complained of hoarseness for 2 years and was found to have laryngeal sarcoidosis with upper airway obstruction.
A 41-year-old non smoking Hispanic female complained of hoarseness of voice for 2 years and was treated for allergic rhinitis for 9 months with no benefit. She also received multiple courses of antibiotics for presumed upper respiratory tract infection and received high dose proton pump inhibitors for 12 months with no effect on hoarseness. Sarcoidosis was diagnosed 13 years ago when chest radiograph showed bilateral hilar lymphadenopathy and transbronchial biopsy revealed non-caseating granuloma. Her disease was characterized by hypercalcemia, hypercalciuria with multiple renal calculi, high angiotensin converting enzyme levels and generalized lymphadenopathy (with non-caseating granuloma on lymph node biopsy). 8 years ago, she was treated with steroids for one year for hypercalcemia and renal calculi. Spirometry and flow volume loops 5 years ago were normal.Examination did not reveal stridor or generalized lymphadenopathy. Spirometry was normal. Flow volume loop showed decrease in peak inspiratory and expiratory flow rates and plateauing of both expiratory and inspiratory loops suggestive of fixed upper airway obstruction. Rigid laryngoscopy under general anesthesia revealed narrowed glottic area and vocal cord rigidity suggestive of an infiltrative process. Biopsies from glottic area, anterior commisure and subglottic area showed granulomatous inflammation consistent with sarcoidosis. AFB was smear negative and bacterial, mycobacterial and fungal cultures were all negative. A diagnosis of laryngeal sarcoidosis with upper airway obstruction was made. 3 months of oral steroids treatment, resulted in marked improvement in hoarseness and flow volume loops. See figures.
Laryngeal involvement has been described in 0.5 to 8.3% of patients with sarcoidosis. It is usually associated with multi-system involvement and only rarely presents as isolated laryngeal disease. Supraglottic and subglottic areas are usually affected and primary vocal cord involvement is rare. The most common presenting features are hoarseness and stridor.Less common symptoms include dysphagia and cough.Laryngoscopy findings include mucosal alterations with erythema and edema, punctate nodules, and mass lesions. The epiglottis is the most frequently affected area, but any portion of the larynx may be involved. Vocal cord dysfunction by direct involvement or Vocal cord paralysis by involvement of the neural pathways, including the nucleus ambiguous, the 10th cranial nerves, and the superior and recurrent laryngeal nerves have also been described. The diagnosis is established by demonstrating granulomatous inflammation on laryngeal biopsy and excluding other causes of granulomatous laryngitis.Treatments used for laryngeal sarcoidosis have included systemic steroids, intralesional steroids, laser resection, surgical excision, low-dose radiation and tracheotomy. Improvement with use of inhaled steroids and Clofazimine (an anti-leprosy agent) has been reported.
In patients with sarcoidosis, persistent hoarseness should prompt an evaluation for laryngeal sarcoidosis by laryngoscopy. Early detection and treatment may prevent development of upper airway obstruction.
Vijay Rupanagudi, None.