Case In Point: A case of bilateral hilar lymphadenopathy

A 38-year-old woman was referred to a clinic for evaluation of newly recognized bilateral hilar lymphadenopathy. She denied any pulmonary or systemic symptoms, including recent fevers, chills, weight loss, dyspnea, cough, and chest pain. Fifteen years earlier, the patient suffered from acute interstitial keratitis and acute hearing loss. A diagnosis of Cogan syndrome was made based on the clinical presentation, an audiogram showing sensorineural deafness, and a negative rapid plasma reagin test for syphilis. Her chest radiograph was normal at that time. Although her symptoms resolved with a course of oral prednisone, she subsequently experienced episodic photosesitivity and blurred vision that responded to treatment with topical corticosteroids. The patient's medical history also included a positive purified protein derivative test result, chronic sinusitis with repeated sinus surgeries, and controlled hypothyroidism. She was an ex-smoker who had smoked cigarettes for 10 years. She had lived in Arizona before moving to California. One year ago, the patient underwent removal of a malignant melanoma. Evaluation at that time included a chest radiograph, which was normal. However, the chest radiograph obtained just before her clinic visit demonstrated new bilateral hilar lymphadenopathy (Figure 1). CT of the chest confirmed bilateral lymphadenopathy in the paratracheal, pretracheal, subcarinal, and hilar regions without parenchymal infiltrates (Figure 2). On physical examination, the patient appeared well with normal vital signs and no fever. There were no palpable supraclavicular or axillary lymph nodes. Examination of the chest, abdomen, and skin did not reveal any abnormalities. Based on the patient's history and age, our greatest concern was for disseminated malignant melanoma or lymphoma. Other likely possibilities included reactivation tuberculosis, pulmonary coccidioidomycosis (because of her residence in the southwestern United States), and pulmonary sarcoidosis (Table). White blood cell count; hemoglobin, calcium, albumin, transaminase, and angiotensin-converting enzyme (ACE) levels; and urinalysis results were all normal. Coccidioides titers indicated no previous exposure. The patient was unable to produce any sputum. Given the absence of pulmonary or systemic symptoms, we decided to obtain nodal tissue for analysis. Mediastinoscopy was performed, and 2 paratracheal lymph nodes were obtained. Noncaseating granulomatous lymphadenitis with giant cells was identified in both lymph nodes (Figure 3). No acid-fast bacilli, fungi, or bacteria were found. A presumptive diagnosis of benign lymphadenopathy secondary to Cogan syndrome was made. The patient continued to do well clinically without treatment 1 year after mediastinoscopy.Discussion Cogan syndrome is a rare disease of young adults that was first defined in 1945 as a clinical entity of audiovestibular symptoms and ocular inflammation.1 Since its first description, 2 clinical presentations have been recognized.2 Typical Cogan syndrome, Cogan syndrome I, is characterized by acute interstitial keratitis with audiovestibular dysfunction (acute bilateral hearing loss, vertigo, tinnitus, nausea, and vomiting). Atypical Cogan syndrome, Cogan syndrome II, is characterized by audiovestibular dysfunction with inflammatory eye lesions in addition to or instead of interstitial keratitis. Systemic involvement has been observed in 20% to 50% of patients with Cogan syndrome.2,3 Lung involvement is very uncommon. Pleuritis (cough, chest pain) and transient chest radiographic