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      Baffling Case of a Patient With History of Lupus in a COVID-19 Pandemic

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          Abstract

          Systemic lupus erythematosus (SLE) is an autoimmune disease with a myriad of clinical presentations and periodic flares. We present a case of a young lady with a history of SLE who presented with constitutional symptoms 1 week after starting Isoniazid and Rifampin for treatment of latent TB. Her presentation shared similarities with several diseases including TB lymphadenitis, SLE flare, Kikuchi-Fujimoto Disease (KFD) and hemophagocytic lymphohistiocytosis (HLH) posing a diagnostic dilemma. Additionally, she presented not long after the onset of the global COVID-19 pandemic, further expanding the differential diagnosis. She was ultimately diagnosed with a severe SLE flare caused by rifampin induced suppression of the CYP3A4 system, thereby reducing the therapeutic efficacy of steroids. This case highlights the deadly potential of drug–drug interactions, especially in patients with autoimmune conditions.

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          Most cited references13

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          HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis.

          In HLH-94, the first prospective international treatment study for hemophagocytic lymphohistiocytosis (HLH), diagnosis was based on five criteria (fever, splenomegaly, bicytopenia, hypertriglyceridemia and/or hypofibrinogenemia, and hemophagocytosis). In HLH-2004 three additional criteria are introduced; low/absent NK-cell-activity, hyperferritinemia, and high-soluble interleukin-2-receptor levels. Altogether five of these eight criteria must be fulfilled, unless family history or molecular diagnosis is consistent with HLH. HLH-2004 chemo-immunotherapy includes etoposide, dexamethasone, cyclosporine A upfront and, in selected patients, intrathecal therapy with methotrexate and corticosteroids. Subsequent hematopoietic stem cell transplantation (HSCT) is recommended for patients with familial disease or molecular diagnosis, and patients with severe and persistent, or reactivated, disease. In order to hopefully further improve diagnosis, therapy and biological understanding, participation in HLH studies is encouraged.
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            Reactive hemophagocytic syndrome in adults: a retrospective analysis of 162 patients.

            Current knowledge in reactive hemophagocytic syndrome mainly relies on single-center case series including a relatively small number of patients. We aimed to identify a multicenter large cohort of adult patients with reactive hemophagocytic syndrome and to describe relevant clinical and laboratory features, underlying conditions, and outcome.
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              Enigmatic Kikuchi-Fujimoto disease: a comprehensive review.

              To determine the clinicopathologic significance of Kikuchi-Fujimoto disease (KFD) and review the literature on this condition, we conducted a MEDLINE search of English-language articles published between 1972 and December 2003. KFD has a worldwide distribution, and Asiatic people have a higher prevalence. Its pathogenesis remains controversial. Patients are young and seek care because of acute tender, cervical lymphadenopathy and low-grade fever. Histologic findings include paracortical areas of coagulative necrosis with abundant karyorrhectic debris. Karyorrhectic foci consist of various types of histiocytes, plasmacytoid monocytes, immunoblasts, and small and large lymphocytes. There is an abundance of T cells with predominance of CD8+ over CD4+ T cells. Differential diagnosis includes lymphoma, lymphadenitis associated with systemic lupus erythematosus, and even adenocarcinoma. KFD is an uncommon, self-limited, and perhaps underdiagnosed process with an excellent prognosis. Accurate clinicopathologic recognition is crucial, particularly because KFD can be mistaken for malignant lymphoma.
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                Author and article information

                Journal
                J Community Hosp Intern Med Perspect
                J Community Hosp Intern Med Perspect
                Journal of Community Hospital Internal Medicine Perspectives
                Greater Baltimore Medical Center
                2000-9666
                2022
                04 July 2022
                : 12
                : 4
                : 49-52
                Affiliations
                [a ]Department of Internal Medicine Greater Baltimore Medical Center, Towson, MD, USA
                [b ]Department of Pulmonary and Critical Care Medicine Greater Baltimore Medical Center, Towson, MD, USA
                Author notes
                [* ]Corresponding author. E-mail address: fhalilu@ 123456gbmc.org (F. Halilu).
                Article
                jchim-12-04-049
                10.55729/2000-9666.1063
                9533801
                36262906
                1dcb58aa-d853-46bc-991e-1c001b50a2ca
                © 2022 Greater Baltimore Medical Center

                This is an open access article under the CC BY-NC license ( http://creativecommons.org/licenses/by-nc/4.0/).

                History
                : 31 January 2022
                : 23 February 2022
                Categories
                Case Report

                systemic lupus erythematosus,kikuchi-fujimoto disease,lymphadenopathy,hemophagocytic lymphohistiocytosis,latent tuberculosis

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