ABSTRACT
INTRODUCTION: Known causes of granulomatous inflammation must be excluded before the diagnosis of sarcoidosis can be secured. We explored the possibility that connective tissue diseases (CTDs) could be a cause of granulomatous inflammation through an analysis of patients cared for in 2 medical centers and a review of the medical literature. METHODS: Patients with both a diagnosis of sarcoidosis and a CTD were identified at two medical centers. In addition, a literature search identified reported cases of patients with both diagnoses. RESULTS: We identified 15 patients at 2 medical centers plus 53 previous reported patients in the medical literature (total=68) with diagnoses of both sarcoidosis and a CTD. The patients were predominantly female. Scleroderma was the most common CTD. Only 2/59 (3%) with chest radiographs recorded had fibrocystic (stage 4) disease. In 48/67 (72%) patients where the time of diagnosis was recorded, sarcoidosis was diagnosed simultaneously or after the CTD. Sarcoidosis in only one organ ("isolated sarcoidosis") was associated with sarcoidosis being diagnosed simultaneously or after the CTD (p=0.0001). CONCLUSIONS: These data suggest that a significant portion of patients with CTDs and sarcoidosis may actually not have the latter disease. Rather, the CTD may "cause" granulomatous inflammation. Alternate explanations for these findings include that sarcoidosis and/or the connective tissue disease was misdiagnosed in these patients. The diagnosis of a concomitant connective tissue disease and sarcoidosis must be made with extreme caution.
Subject(s)
Connective Tissue Diseases/diagnosis , Sarcoidosis, Pulmonary/diagnosis , Connective Tissue Diseases/complications , Diagnosis, Differential , Diagnostic Errors , Female , Granuloma, Respiratory Tract/diagnosis , Granuloma, Respiratory Tract/etiology , Humans , Middle AgedABSTRACT
Sarcoidosis is a common disease and affects the respiratory system in > 90% of cases, most commonly the intrathoracic lymph nodes and the respiratory parenchyma. Less commonly, the airways are involved, and the disease is manifested as mucosal erythema, edema, granularity and cobblestoning, plaques, nodules, and bronchial stenosis, airway distortion, traction bronchiectasis, and bronchiolitis. Airway involvement may lead to airflow limitation. Involvement of oral, nasal, and pharyngeal mucosa may cause hoarseness, dysphagia, laryngeal paralysis, and upper airway obstruction. Airway symptoms are important indicators of airway involvement in sarcoidosis. Pulmonary function testing, radiologic imaging, and bronchoscopy occupy a significant role in the diagnosis and management of airway involvement in patients with sarcoidosis.
Subject(s)
Sarcoidosis, Pulmonary/physiopathology , Airway Obstruction/etiology , Airway Obstruction/pathology , Airway Obstruction/physiopathology , Bronchi/pathology , Bronchi/physiopathology , Bronchiectasis/pathology , Bronchiectasis/physiopathology , Bronchoscopy , Hemoptysis/etiology , Hemoptysis/pathology , Hemoptysis/physiopathology , Humans , Laryngeal Diseases/etiology , Mucous Membrane/pathology , Mucous Membrane/physiopathology , Sarcoidosis, Pulmonary/diagnosis , Sarcoidosis, Pulmonary/pathology , Sleep Apnea, Obstructive/etiology , Trachea/pathology , Trachea/physiopathologyABSTRACT
Wegener's granulomatosis is characterized by necrotizing granulomatous inflammation and necrotizing vasculitis affecting predominantly small arteries, arterioles, capillaries, and venules. In contrast to the well-described pulmonary parenchymal involvement of Wegener's granulomatosis, the lower airway (tracheobronchial) disease manifestations are less well recognized by clinicians. Consequently, mild disease of the airways is easily missed. There is a relative paucity of published information on various tracheobronchial manifestations of Wegener's granulomatosis. This article provides a comprehensive review of the diagnosis and management of the infraglottic tracheobronchial disease manifestations.
