The role of transbronchial lung biopsies (TBB) in the diagnostic workup of systemic inflammatory rheumatic disease-associated interstitial lung disease (SIRD-ILD) is unclear and TBB is not generally recommended. The study objective was to examine the utility of TBB to guide treatment in a population of patients with SIRD-ILD. All patients from the Department of Rheumatology, Rigshospitalet, Denmark, who had TBB performed, from 2002 to 2016 were identified. Patient demographics as well as smoking status, previous lung disease, pulmonary function test, SIRD-diagnosis, imaging results and immunomodulatory therapy pre- and post-bronchoscopy were obtained. Histology findings were used to dichotomize patients into a high-inflammatory group or a low-inflammatory group. The high-inflammation group primarily consisted of non-specific interstitial pneumonia, organizing pneumonia, lymphocytic infiltrating pneumonia and granulomatous inflammation whereas the low inflammation group primarily consisted of histological findings of usual interstitial pneumonitis and biopsies describing fibrosis and/or sparse unspecific inflammation. Therapeutic consequence was defined as intensification of therapy. Differences in treatment intensification were calculated using a binominal logistic regression model. Ninety-six patients had TBB performed. Biopsies from 55 patients were categorized as high inflammatory and 41 as low inflammatory, respectively. In the high-inflammatory group, 38 (69%) had their therapy intensified compared to 6 (14%) in the low-inflammatory group (Odds ratio 8.0, 95% confidence limits 3.2-20.0, P < 0.001). No procedure-related complications were registered. TBB findings can guide treatment strategy in SIRD-ILD patients with suspected activity in the pulmonary disease. TBB appears safe and could be considered as part of the diagnostic workup.