Interstitial lung diseases are a group of disorders with progressive, unrelenting course. Of which, Connective tissue disorder (CTD) associated ILD has a better prognosis than the idiopathic group. Connective tissue disorders have varied symptoms and the diagnosis of associated ILD requires a definite diagnosis of underlying CTD. Rarely CTD presents with predominant lung involvement or even absent extra-pulmonary manifestations. This newly recognised entity requires a long term follow up and regular CTD workup to be picked up early. Lung dominant CTD is common in young females and a predominant NSIP pattern on HRCT. Our case 21 year old female, diagnosed with ILD in 2007 followed through to 2019 after which the underlying CTD was diagnosed. It is an uncommon presentation of lung dominant CTD with an unusually long duration after which the underlying CTD was diagnosed. With ILD progression over 15 years and rapid deterioration of pulmonary hypertension which warranted an extensive CTD work-up led to the diagnosis of anti-synthetase syndrome with lung dominant presentation.
• Patients with rapid deterioration in terms of pulmonary hypertension warrants active search and extended work-up of CTD even in the absence of symptoms
• Young females with no exposure to known causes of ILD - CTD features and workup needs to be assessed regularly
• Most commonly idiopathic inflammatory myopathy present with lung predominant symptoms.