PURPOSE: Interstitial lung disease (ILD) may be the first manifestation of connective tissue disease (CTD). Distinguishing ILD associated with CTD from idiopathic interstitial pneumonias may influence management and prognostication. The goal of this study was to establish a profile of the current practice at our Institution in this regard and to determine potential benefits of a standardized approach of patients with newly diagnosed ILD.
METHODS: We retrospectively reviewed medical records of patients seen in our ILD clinic over a 2-year period from 01/01/2001 to 12/31/2002 to assess the incidence of CTD-associated ILD. Collected data included demographics, smoking history, clinical findings, pulmonary function tests, computed tomography of the lungs, bronchoscopy, lung biopsy, renal and liver function tests, urine analysis, muscular enzymes, cell count, sedimentation rate, antinuclear antibodies, antibodies to cyclic citrullinated peptides, and antineutrophil cytoplasmic antibodies.
RESULTS: Of 357 patients with ILD, 56 (15.7%) had CTD. Those diagnosed to have CTD-associated ILD included 33 with a previously known CTD, 23 a newly diagnosed CTD; 5 additional patients had positive serologies only. CTD associated with ILD included 14 sclerodermas, 10 rheumatoid arthritis, 8 poly- or dermatomyositis, 5 mixed CTD, 5 undifferentiated CTD, 3 overlap syndromes, 2 lupus erythematosus, and 9 miscellaneous.
CONCLUSION: Of 357 patients who presented to the ILD clinic, 15% had CTD, 41% of which were newly diagnosed with CTD.
CLINICAL IMPLICATIONS: Screening patients presenting with ILD with serologic markers for CTD can uncover the underlying cause in a significant portion of patients.
DISCLOSURE: Philippe Bauer, No Financial Disclosure Information; No Product/Research Disclosure Information