There is mild bilateral hilar adenopathy. Constitutional symptoms of fever, weight loss and fatigue may be present. Hemoptysis is rare, as is bone involvement in patients with pulmonary manifestations. Pulmonary function tests are non-diagnostic with normal, restrictive, obstructive, or mixed patterns all seen. Presentation at extremes of age, multi-organ involvement, low diffusion capacity and recurrent pneumothoraces are independent poor prognostic indicators.
Treatment is largely symptomatic.
Intervening lung is often normal. Less commonly one may see cavitation in larger nodules, lymphadenopathy, ground-glass opacity or fine reticular opacity. HRCT features may be identical in these patients. TS lung disease appears very similar to LAM but can be seen in males. End-stage Idiopathic pulmonary fibrosis may occasionally cause confusion with extensive basal and subpleural honeycombing but the intervening lung is abnormal and fibrotic.