“…Bronchiolitis obliterans is a rare complication of RA, characterized with a progressive and often fatal clinical course, with widespread obliterative bronchiolitis on CT. D-penicillamine has been associated with bronchiolitis obliterans, although the role of D-penicillamine as the sole etiological agent is uncertain, given the rarity of bronchiolitis obliterans in patients receiving the drug for Wilson's disease. 14 While the clinical syndrome of bronchiolitis obliterans is rare in patients with RA, CT features of obliterative bronchiolitis are relatively common and are often subclinical. 15 Areas of decreased attenuation on HRCT are present in two-thirds of patients with newly diagnosed RA in one study, in 18% of cases of more than 20% extent.…”
Pulmonary disease represents a significant extra-articular manifestation in the majority of connective tissue diseases (CTDs). The identification, classification, and staging of pulmonary involvement are centrally important to the management of patients, aiding the prognostication of disease behavior and treatment decisions. We present a review of the high-resolution computed tomographic pulmonary features of CTD in the lung and their significance to the reporting radiologist.
“…Bronchiolitis obliterans is a rare complication of RA, characterized with a progressive and often fatal clinical course, with widespread obliterative bronchiolitis on CT. D-penicillamine has been associated with bronchiolitis obliterans, although the role of D-penicillamine as the sole etiological agent is uncertain, given the rarity of bronchiolitis obliterans in patients receiving the drug for Wilson's disease. 14 While the clinical syndrome of bronchiolitis obliterans is rare in patients with RA, CT features of obliterative bronchiolitis are relatively common and are often subclinical. 15 Areas of decreased attenuation on HRCT are present in two-thirds of patients with newly diagnosed RA in one study, in 18% of cases of more than 20% extent.…”
Pulmonary disease represents a significant extra-articular manifestation in the majority of connective tissue diseases (CTDs). The identification, classification, and staging of pulmonary involvement are centrally important to the management of patients, aiding the prognostication of disease behavior and treatment decisions. We present a review of the high-resolution computed tomographic pulmonary features of CTD in the lung and their significance to the reporting radiologist.
S Penicillamine Bronchiolitis obliterans: case reportA 47-year-old man developed bronchiolitis obliterans during treatment with penicillamine for hepatolenticular degeneration (Wilson's disease).The man had a history of hypertension, dyslipidaemia treated with statins, Graves-Basedow disease treated with replacement therapy; he also had Wilson's disease, diagnosed 20 years earlier, for which he had received penicillamine for the previous 2 years [dosage and route not stated]. He presented with dyspnoea on exertion and an initial lung function study showed severe airflow limitation.Inhaled corticosteroids were initiated. Complete lung function testing showed severe irreversible obstructive changes with air trapping and reduced carbon monoxide diffusing capacity. A chest CT scan revealed discrete bronchiectasis and diffuse bronchiolectasis. A sweat test excluded cystic fibrosis and an immunoglobulin study was normal. Respiratory failure was observed during a follow-up visit and the man was admitted for further tests. Examination revealed an oxygen saturation of 88% on room air, without laboured breathing, and normal pulmonary auscultation. As drug-induced bronchiolitis was suspected, a lingular biopsy was obtained via anterior thoracotomy. He was subsequently discharged with domiciliary oxygen. The final diagnosis from the pathological study was follicular bronchiolitis associated with constrictive bronchiolitis obliterans. Penicillamine was withdrawn and he began receiving bronchodilators and methylprednisolone. He subsequently underwent a successful double lung transplant.Author comment: "To conclude, this clinical case is presented as an example of an infrequent but previously described side effect of a treatment [penicillamine] which is commonly used in several diseases." Bruguera-Avila N, et al. Obliterating bronchiolitis in a patient treated with (D)penicillamine. Archivos de Bronconeumologia 49: 411-2, No. 9,
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