Ro-positive interstitial lung disease treated with cyclophosphamide
Bedwani, Nader Habib Jefferson, Natasha Marguerie, Christopher Mukherjee, Jayanta
Bedwani, Nader Habib
Jefferson, Natasha
Marguerie, Christopher
Mukherjee, Jayanta
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Affiliation
South Warwickshire University NHS Foundation Trust
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Publication date
2018-05-03
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Abstract
Interstitial lung disease (ILD) comprises a spectrum of conditions involving inflammation and/or fibrosis of the alveolar wall causing limitation in gaseous exchange. Treatment varies depending on the underlying ILD. We describe the case of a woman presenting with a productive cough who was diagnosed with community-acquired pneumonia. While on the ward she developed type-1 respiratory failure requiring continuous positive airway pressure and intensive care unit admission. Failing to respond to targeted antimicrobials she was investigated by chest high-resolution CT and autoantibody screen to identify non-infective causes of her respiratory signs and symptoms. These demonstrated diffuse ground-glass change with peripheral honeycombing in keeping with fibrosis and alveolitis alongside high titres of anti-SS-A/Ro antibodies. She was managed with reducing course of steroids and immunosuppression with cyclophosphamide. The rational of long-term immunosuppression was based on a presumed diagnosis of lung-dominant connective tissue disease, a disease concept proposed in contemporary medical literature.
Citation
Habib Bedwani N, Jefferson N, Marguerie C, Mukherjee J. Ro-positive interstitial lung disease treated with cyclophosphamide. BMJ Case Rep. 2018 May 22;2018:bcr2017223925. doi: 10.1136/bcr-2017-223925.
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