Abstract We present the case of a 62 year old woman with past medical history of Asthma and smoking, who had presented to our hospital for abdominal pain and dyspnea on exertion. On history, the patient had been seen in the emergency department several times prior to this admission for abdominal pain and dyspnea on exertion, and had been admitted to another hospital for acute hypoxic respiratory failure in setting of multifocal pneumonia. She had most recently been discharged on home oxygen due to persistent hypoxia while being hospitalized. During this time, she had had multiple courses of broad spectrum antibiotics and steroids for multifocal pneumonia and suspected superimposed asthma exacerbation. Imaging during this time showed persistent multifocal airspace disease process. At current presentation to our emergency department, CT images showed evidence of abdominal mass and multifocal consolidative and pleuro-fibrotic processes bilaterally, which were unchanged from prior. Given appearance and chronicity, urgent biopsies were obtained of the abdominal mass and consolidative process to obtain tissue diagnosis. Abdominal mass biopsy showed evidence of retroperitoneal fibrosis, and lung biopsy showed evidence of organizing pneumonia. Rheumatology was involved, along side pulmonology, and multi-specialty approach was applied to reach a diagnosis of diagnosis of IgG-4 related disease spectrum with multi system involvement. The patient was started on mycophenolate 1000 mg twice daily, and chronic low dose prednisone adjusted to abdominal pain, and more recently was started on Inebilizumab infusion by rheumatology. Repeat CT images has shown persistent but improved airspace disease, with further development of more commonly reported finding of bronchiectasis as part of IgG-4 related disease spectrum. IgG-4 related disease spectrum is a rare disease process to begin with, and pulmonary involvement, albeit documented, is rare. More commonly reported lung findings include solid nodules, broncho-vascular findings such as interlobular septal thickening, alveolar interstitial findings such as honeycombing, bronchiectasis and diffuse ground glass opacities, and round shaped ground glass opacities. In the literature, organizing pneumonia has been documented as one of the potential pulmonary findings for IgG-4 related disease spectrum, if present. We aim to add to the literature by reporting on organizing pneumonia as part of the pulmonary manifestations of IgG-4 related disease spectrum, in order to help expand our understanding on this complex disease entity. This abstract is funded by: none
Ashfaq et al. (Fri,) studied this question.