Published online by Cambridge University Press: 29 June 2007
Two cases with laryngeal manifestations of systemic lupus erythematosus presented demonstrate the value of serial indirect laryngeal photography and results of treatment for the mucosal and serosal effects of the disease.
Systemic lupus erythematosus is an autoimmune collagen vascular disease which produces widespread damage to connective tissue, blood vessels, serosal surfaces and mucous membranes (Rodman et ah, 1973). It is no surprise that with such a widespread disorder the upper airways too may show involvement.Estimates of the number of patients with laryngeal involvement have ranged from one percent by Dubois (1966) to one-third of patients by other authors (Babich and Tranaov, 1970; Minchina et al., 1971).
Systemic lupus erythematosus may cause laryngeal symptoms referrable to an active mucosal, submucosal or serosal process. With acute flares of the disease, mucosal ulceration, edema, and submucosal hematomas may result in hoarseness and throat pain (Scarpelli et al., 1959). One case of epiglottitis caused by increased mucosal swelling has been reported (Toomey et al., 1974). As the disease progresses, late effects of mucosal disease including corditis, mucosal thickening, laryngeal scarring with stenosis and laryngitis with dry, thickened vocal cords may appear (Babich and Tramaov, 1970; Smith and Ferguson, 1976). With serosal involvement, the patient may demonstrate perichrondritis, cricoarytenoid arthritis or actual vocal cord paralysis (Dubois, 1966; Montgomery and Lofgren, 1963).
Histopathologic study of involved mucosa along with cultures of the tissue is not diagnostic of systemic lupus erythematosus but can help to rule out other disorders similar in appearance such as tuberculosis and rhinoscleroma. Klebsiella rhinoscleromata infections particularly can cause a similar early inflammatory mucosal and late scarred picture.
Microscopic sections of mucosa from a patient who died of laryngeal edema secondary to systemic lupus erythematosus appeared similar to mucosa from a patient with subglottic stenosis (Smith and Ferguson, 1976) with an infiltration of histiocytes, lymphocytes, plasma and mast cells.
The following two cases demonstrate the differences in the course of serosal and mucosal airways disease.