OP0081 A CASE OF ATYPICAL MYCOBACTERIUM INFECTION COMPLICATING EXTRA-NODAL ROSAI-DORFMAN DISEASE IN A PATIENT WITH SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)
Authors: K. Su, G. Hagan, S. Tosounidou, C. Gordon, J. Reynolds
Journal: Annals of the Rheumatic Diseases (2021)

Background: We present the case of a 28 year old Black-British female with severe SLE requiring treatment with rituximab in 2012 due to persistent low-grade activity and severe episodes of pleuro-pericardial effusions, pancytopaenia, fever and weight loss Her other background includes beta-thalassaemia trait and excision of calcific fibrotic tissue on bilateral anterolateral orbits in 2015. In 2018 she reported an 18-month history of non-tender, non-fluctuant, slow growing left thigh mass with USS revealing a well demarcated subcutaneous complex cystic lesion of ~2x4x7cm. There was no preceding trauma or skin infection. Histology from a needle biopsy revealed diffuse histiocytosis with positive immunohistochemistry (ICH) for S100, CD68 and CD31, it was negative for CD1a, consistent with Extra-nodal Rosai-Dorfman disease (RDD ). She developed constitutional symptoms after reporting months of gradual weight loss with gradual ESR, CRP rise and leucocytosis. Her SLE symptoms were stable and given lack of SLE-specific symptoms; PET-CT was used to identify systemic RDD; the thigh mass showed strong FDG avidity along with a small focus of uptake in the small bowel, thought to be RDD related with no other areas of uptake. She had ongoing ooze from the enlarging thigh lesion (5 x 26 x 15 cm), this was sent for MCS and AAFB; which isolated Mycobacterium avium . She was treated with rifampicin, ethambutol and clarithromycin resulting in improved thigh lesion, constitutional symptoms and inflammatory markers. Objectives: [1]To describe a rare associated complication of severe SLE and to educate and inform clinicians regarding possible masquerades of disease [2]To education and inform about the approach to diagnosis of mycobacterium infection. Methods: Case report and literature review. Results: Mycobacterium infections rarely complicate RDD; to date, only one case report is published involving an HIV infected patient with RDD confirmed on LN biopsy presenting with splenomegaly and treated with oral corticosteroids (OCS) complicated by Mycobacterium avium complex and Salmonella enterica confirmed on bone marrow biopsy/culture, similar to our patient, he presented with constitutional symptoms and weight loss (2) . Mycobacterium can also mimic RDD, a case report has described a 74 year old with tender lymphadenopathy diagnosed with RDD on LN biopsy. She was treated with IV and OCS, but was unresponsive. A repeat LN biopsy and CT imaging revealed the presence of mycobacterium kansasii; her biopsy was positive for CD68/S100 throughout. Of note, she had high levels of anti-interferon autoantibodies and was diagnosed with adult-onset immunodeficiency syndrome (3) . Conclusion: This case illustrates the need for a MDT approach for multi-system diseases such as SLE and RDD, and the need to consider atypical infections when blood tests are incongruent with clinical state. References: [1]Bruce-Brand C, Schneider JW, Schubert P. Rosai-Dorfman disease: an overview. J Clin Pathol. 2020 Nov;73(11):697-705. doi: 10.1136/jclinpath-2020-206733. [2]Olmedo-Reneaum A, Molina-Jaimes A, Conde-Vazquez E, Montero-Vazquez S. Rosai-Dorfman disease and superinfection due to Salmonella enterica and Mycobacterium avium complex in a patient living with HIV. IDCases. 2020 Jan 14;19:e00698. [3]King YA, Hu CH, Lee YJ, Lin CF, Liu D, Wang KH. Disseminated cutaneous Mycobacterium kansasii infection presenting with Rosai-Dorfman disease-like histological features in a patient carrying anti-interferon-γ autoantibodies. J Dermatol. 2017 Dec;44(12):1396-1400. Image 1. Table 1. SLE Clinical History Diagnosed 2006 (‘97 ACR Classification Criteria) Clinical -Polyarthritis -Glandular (lacrimal swelling) -Pericardial effusion/Pleural Effusion -Myositis Serological -Anti-nuclear antibody (ANA) -Anti-dsDNA -Anti-U1-RNP -Anti- SS-A/Ro -Lupus Anticoagulant Previous SLE Treatment -Hydroxychloroquine (HCQ) -Methotrexate (MTX) -Azathioprine (AZA) -Rituximab (RTX) Disclosure of Interests: None declared