The spectrum of connective tissue diseases (CTDs) includes a heterogeneous group of disorders characterized by circulating autoantibodies and organ-specific autoimmune injuries, leading to variable systemic manifestations. Diffuse parenchymal lung diseases (DPLDs) are the most common pulmonary manifestation, and a baseline assessment of morphological patterns and ongoing pathogenic activity have relevant implications in terms of prognostic estimates and therapeutic management. Moreover, in some cases, when clinical, serological, or morphological features do not meet criteria for a specific rheumatic disease, tissue acquisition gains, even more, a relevant role, as the identification of morphological pattern is crucial to estimate patient prognosis and guide management.
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