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A1573 - A Cure or a Curse? Severe Bilateral Radiation Pneumonitis in a Patient with Scleroderma-Related Interstitial Lung Disease and Lung Adenocarcinoma
Author Block: P. Khaing, R. S. Summer; Pulmonary and Critical Care Medicine, Sidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, PA, United States.
Introduction: Radiation-induced lung injury (RILI) classically presents with radiographic abnormalities confined to the field of radiation. However, certain patient factors such as preexisting interstitial lung disease (ILD) increase the overall risk of RILI and may be associated with uncharacteristic clinical manifestations. Case: A 78 year-old man with scleroderma-related ILD presented to the emergency room with several weeks of dyspnea, dry cough, and weight loss. Approximately seven weeks ago, he had completed therapy for stage IIb lung adenocarcinoma with carboplatin/paclitaxel and concurrent radiotherapy (total 60 gray via volumetric modulated arc therapy (VMAT) to right lower lobe). He reported being on a taper of prednisone for radiation esophagitis, but otherwise, denied any smoking history, sick contacts, recent travel or new medications. Vital signs showed an oxygen saturation of 84% on room air. Physical exam was notable for coarse bibasilar crackles and skin tightening of the face and hands. Labs were unremarkable. A positron emission tomography (PET) scan, completed the day of presentation, showed hypermetabolic areas of diffuse ground glass opacities and consolidations that were located in the bilateral lower lung zones. The patient was empirically treated with antibiotics and diuretics, but infectious and cardiac etiologies were ruled out. Bronchoalveolar lavage showed a mixed neutrophilic and lymphocytic predominance with negative cultures. The patient was diagnosed with severe bilateral RILI in setting of scleroderma-related ILD. High dose corticosteroids were started but there was no clinical improvement. A week later, repeat imaging showed progression of disease with diffuse parenchymal scarring and extensive traction bronchiectasis in both lower lung zones. There was also new pneumomediastinum and subcutaneous emphysema, which was attributed to alveolar rupture from extensive tissue destruction, after ruling out esophageal perforation with a barium swallow. After a prolonged hospitalization, the patient was eventually discharged home on supplemental oxygen. Currently, he is receiving oral mycophenolate and rituximab infusions but continues to have severe respiratory compromise. Discussion: The existing medical literature contains a large gap in knowledge regarding the true risk of radiation in patients with ILD, especially in those with connective tissue disorders. Our patient with preexisting scleroderma-related ILD, who received chemo-radiotherapy for lung adenocarcinoma, developed extensive lung injury that extended beyond the field of radiation and progressed to extensive and irreversible tissue destruction despite high dose corticosteroids. Understanding the risks of chemo-radiotherapy in patients with ILD is crucial for physicians and patients to make well-informed shared medical decisions, but more information is needed.