Ocular, aural, and oral manifestations of lupus

Eye Systemic lupus erythematosus (SLE) may affect any organ or tissue in the body, and the eye is no exception. Although the human eye measures less than 3 cm from cornea to retina, the eye contains a diverse array of structures, almost any of which can be the target of inflammation. The manifestations of SLE in the eye are therefore varied and range from dry eye to infiltrative keratitis, scleritis, episcleritis, retinal vasculitis, optic neuropathy, and orbital inflammation. The eyelid can be involved in cutaneous lupus, and ocular motility can be affected by cranial nerve abnormalities or by orbital myositis. The most common of the ocular manifestations is dry eye or keratoconjunctivitis sicca (KCS) as a result of secondary Sjögren’s (Chapter 32 discusses this topic in detail). Of the other ocular pathologic conditions, retinal vasculopathy in the form of cotton wool spots is the next most common and has ominous systemic implications. Optic neuropathy, although rare, is associated with a poor visual prognosis. Less common manifestations are also briefly discussed in this section. In addition, this chapter discusses the known side effects of antimalarial drugs-a class of common medications used in SLE. Mouth Oral findings in lupus are common but understudied relative to other manifestations of disease. Oral ulcers are a criterion in the main classification systems with prevalence estimates varying widely from 7% to 54%, in part due to varying rigor in examining the oral mucosa, the asymptomatic nature of some lesions, and reporting biases. The classical lesions in systemic lupus erythematosus (SLE) are painless ulcers of the hard palate and in chronic cutaneous lupus erythematosus (CCLE) are discoid lesions of the buccal mucosa, lips, or soft palate. However, less common findings include verrucous lesions, bullous lesions, and cheilitis. An uncomplicated oral lupus ulcer can be diagnosed on the basis of clinical appearance alone with a biopsy adding useful information in only a minority of cases and usually when other diseases are suspected. Other oral manifestations result from chronic ulcers, medications used to treat lupus, and diseases associated with lupus. Chronic inflammation from persistent ulceration predisposes to malignancy. Medications used to treat lupus can have toxic and infectious complications affecting the mouth. Sjögren’s results in sicca symptoms. Finally, common conditions affecting the oral mucosa can occur in patients with lupus and these mimickers need to be considered in atypical cases. Nose The presence of nasal ulcers is included as one of the criterions in the SLICC Classification Criteria although reports on the spectrum of disease are limited. Some series report almost half of patients having nasal symptoms or mucosal abnormalities on exam although other series report much lower rates. Common symptoms include congestion, pruritus, dryness, crusting, and epistaxis. When ulcers are present, they tend to relapse and remit without intervention. Topical corticosteroids may be helpful in recurrent or refractory disease. Nasal septal perforation has been reported and can be asymptomatic. Mimickers of nasal lupus include nasal trauma (nose picking) and cocaine. Vasculitis secondary to SLE or as a primary condition should be considered in particularly aggressive cases. Larynx Laryngeal involvement of lupus is uncommon. However, case reports exist for acute epiglottitis, mucosal ulceration, laryngeal edema, laryngitis sicca, chronic hyperplastic laryngitis, cricoarytenoid arthritis, and vocal cord paralysis. Patients with laryngeal inflammation may present with dysphonia, hoarseness, a sore throat, and/or a chronic cough. Persistent symptoms should prompt further work-up by an otolaryngologist. Ear Sensorineural hearing loss can be a form of autoimmune vestibulitis and is noted in less than 1% with SLE. Early treatment with anti-inflammatory regimens is critical. Vertigo may be associated with a Meniere’s like picture.