Abstract Introduction Extraintestinal manifestations (EIM) of inflammatory bowel disease (IBD) are well described and commonly involve multiple organ systems. Identification of EIM of IBD is typically secondary to clinical symptoms. Rarely, the initial presentation of IBD includes pulmonary EIM such as organizing pneumonia, pulmonary granulomatous disease, and interstitial lung disease. IBD treatment includes various immunosuppressive agents that increase the risk of opportunistic infection, which complicates determining the etiology of pulmonary EIM of IBD. We present the first known case of a child who developed necrobiotic pulmonary nodules as EIM of Crohn’s Disease (CD) following upadacitinib induction and initiation of maintenance therapy. Description of Case A 13-year-old female with CD, sacroiliitis, psoriasis, and episcleritis, who recently transitioned to upadacitinib from infliximab, presented with frank hematuria two days after falling. Six weeks prior, upadacitinib induction was completed with improvement of gastrointestinal symptoms of CD and psoriasis; however, within one week she noted increased gastrointestinal and ocular symptoms consistent with prior CD flares on dose reduction. Abdominal computed tomography (CT) revealed a left ureteral non-obstructing stone, but no evidence of traumatic kidney injury. Additional findings included a region of possible enteritis of the terminal ileum and multiple noncalcified nodular densities in bilateral lung bases. Chest radiograph suggested only lower lobe nodules. Chest CT revealed multiple randomly distributed pulmonary nodules most numerous in bilateral lower lobes and the largest in left upper lobe, some of which were cavitary. Despite these findings, the patient had no symptoms of pulmonary disease. Bronchoalveolar lavage did not reveal active bacterial, viral or fungal infection. Immune evaluation revealed negative autoantibodies, normal complement levels, and normal IgG subclass 4. Histopathology of fine-needle biopsy revealed necrobiotic nodules composed of necrotizing granuloma with associated fibrin, surrounding histiocytes, and focal giant cells consistent with pulmonary EIM of CD. The patient completed a five-week course of tapered high-dose systemic corticosteroids. Repeat chest CT revealed substantial reduction in size and number of nodules. Discussion This is the first known case of a child developing necrobiotic pulmonary nodules as EIM of CD during treatment with a JAK-inhibitor. Notably, this finding was incidental, as the patient was without pulmonary symptoms. This case demonstrates that the absence of pulmonary symptoms does not exclude the presence of pulmonary EIM of IBD. Routine lung surveillance is not recommended for patients with IBD; however, subclinical EIM of IBD should be considered in children with disease flares as these findings impact clinical management. This abstract is funded by: None
Magier et al. (Fri,) studied this question.