A 55-year-old black woman presents with fatigue, nonproductive cough, and dyspnea when walking two blocks on level ground or climbing twelve steps for the past three months. She has mild pain but no swelling in the small joints of her hands. She has no history of fever, night sweats, or chest pain, has never smoked, and has worked many years as a front desk secretary in a physician's office. She has no pets at home and is not taking any medications. A new chest x-ray and CT scan show upper lobe predominant ground-glass opacities, and bilateral hilar and subcarinal lymphadenopathy. PFTs today show TLC = 70% predicted, and DLCO = 65% predicted. What is the best test to confirm the diagnosis?
a. Bronchoscopy with transbronchial lung biopsy
b. Lymphocyte proliferation testing of her blood or bronchoalveolar lavage fluid
c. Peripheral blood or bronchoalveolar lavage eosinophilic count
d. Serological studies for connective tissue disease
e. Video-assisted thoracoscopic surgery (VATS) biopsy
The most correct answer is a, bronchoscopy with transbronchial lung biopsy.
The patient presents with classic clinical features of sarcoidosis, including fatigue and joint pains. The adjusted annual incidence among black Americans is roughly three times that among white Americans. The patient has radiological Stage III pulmonary sarcoidosis, and restrictive ventilatory limitation with impairment of the DLCO. Unlike idiopathic interstitial pneumonias, bronchoscopy with transbronchial biopsy confirms the presence of non-necrotizing granulomatous inflammation. In most cases, VATS is not warranted. Her overall clinical presentation and radiological features are not suggestive of eosinophilic lung diseases; hence, the eosinophilic count is not helpful in arriving at a diagnosis. Although the patient has mild joint pain or arthralgia, she does not have any joint swelling or other evidence of a connective tissue disease.
A 48-year-old white man with a history of severe depression presents with shortness of breath and nonproductive cough of 1-year duration. He has smoked two packs of cigarettes daily since 16 years old, but has no significant occupational exposures. He denies joint pains, skin rashes, fever, loss of weight or appetite, or chest pain. Physical exam reveals bilateral basilar crackles. A chest CT scan shows diffuse ground-glass appearance in middle and lower lung zones with reticular infiltrates in the bases. A previous chest x-ray from 2 years ago revealed mild reticular markings at the bases of the lungs. Pulmonary function tests indicate FVC = 69%, FEV1 = 68%, FEV1/FVC = 84%, TLC = 65%, and DLCO = 64%, all of predicted. Bronchoscopy with bronchoalveolar lavage is unremarkable. Which of the following is most likely to improve this patient's disease?
b. Cytotoxic therapy with cyclophosphamide
d. Pulmonary rehabilitation
The most correct answer is e, smoking cessation.
The patient has smoking-related interstitial lung disease. There is nothing in the history to suggest that he is immunocompromised, and there is no other organ involvement to suggest the presence of connective tissue disuse. His normal bronchoalveolar lavage reduces the likelihood of an infectious etiology, and it also excludes diffuse alveolar hemorrhage and eosinophilic lung diseases. The patient appears to have chronic ILD based on the radiological features, while his younger age and the ground-glass appearance on CT scan are not consistent with IPF but are rather suggestive of either desquamative interstitial pneumonia or respiratory bronchiolitis interstitial lung disease. These two diseases are similar clinically, and both have a good prognosis with smoking cessation.