Correct!
3. Discontinue pirfenidone therapy and consult rheumatology
Pirfenidone therapy has recognized benefits for reducing lung function decline in patients with usual interstitial pneumonia/idiopathic pulmonary fibrosis, but that diagnosis has not been established for this patient and therefore this therapy is not appropriate for this patient. The presence of an NSIP pattern at thoracic CT and the positive antibody testing raises concern for a connective tissue disorder, and therefore, consultation with rheumatology is appropriate. Right heart catheterization could be considered given the relatively disproportionately low DLCO compared with the FVC, but typically the investigation of pulmonary hypertension would start non-invasively with echocardiography. Bronchoscopy may provide useful information in the evaluation of patients with diffuse lung disease, occasionally providing the correct diagnosis, or at least excluding some competing causes, particularly infections, that would be inappropriately treated should immunosuppressive therapy be employed. Cryobiopsy may provide tissue for histopathological sampling and could allow a definitive diagnosis for the fibrotic lung disease in this patient, but the patient’s clinical presentation and thoracic CT findings raise the possibility of a systemic disorder rather than an idiopathic interstitial pneumonia, and it is therefore unlikely that lung tissue sampling would provide information that will alter or substantially impact therapy. 133 Xe-Ventilation – 99m Tc-perfusion scintigraphy would be useful to assess for acute or chronic thromboembolic disease or possibly to assess for relative pulmonary perfusion prior to thoracic surgery, but these considerations or probably not appropriate for this patient. The reduced DLCO does raise concern for pulmonary vascular disease, but the previous thoracic CT was an enhanced study and showed no evidence of acute or chronic pulmonary emboli. It would be highly unlikely that 133 Xe-Ventilation – 99m Tc-perfusion scintigraphy would reveal high-probability results in the context of underlying structural lung disease with a recent enhanced thoracic CT examination showing no evidence of pulmonary emboli.
Upon further questioning and examination, the patient did note intermittent finger swelling for the previous 2 years and had intermittent symptoms consistent with Raynaud’s phenomenon. No skin ulceration was present, but skin thickening and hardening over the fingers and hands, as well as subcutaneous tissue atrophy, was present, consistent with sclerodactyly.
Which of the following represents the most likely diagnosis for this patient? (Click on the correct answer to proceed to the sixth of eight pages)