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      The Differential Diagnosis of Bilateral Pleural Effusion and Multiple Mediastinal Lymphadenopathies Includes Kikuchi-Fujimoto Disease

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          Abstract

          Kikuchi-Fujimoto disease is an uncommon lymphohistiocytic disorder that frequently presents with acute or subacute clinical disease course. Cervical lymphadenopathy is the most common involved lymph node. Very rare cases of pathologic diagnosis of Kikuchi-Fujimoto disease with bilateral pleural effusion and multiple mediastinal lymphadenopathies have been reported in the literature. In this article, we report the case of a 60-year-old male presented with bilateral pleural effusion and multiple mediastinal lymphadenopathies. He received video-assisted thoracoscopic surgery of the right pleura and thoracoscopic excision of the mediastinal lymph node. The pathologic findings from the lymph node and pleura were compatible with Kikuchi-Fujimoto disease. He was treated with oral hydroxychloroquine and oral prednisolone. A computed tomography scan of the chest 4 months later showed regressive mediastinal lymphadenopathy and bilateral pleural effusion. Our case is a first reported case of Kikuchi-Fujimoto disease diagnosis by the pathology of the lymph node and pleura in the literature. Results from our case suggest that Kikuchi-Fujimoto disease should be included in the differential diagnosis of bilateral pleural effusion and multiple mediastinal lymphadenopathies.

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          Kikuchi's histiocytic necrotizing lymphadenitis: an analysis of 108 cases with emphasis on differential diagnosis.

          Kikuchi's necrotizing lymphadenitis has now become recognized in many parts of the world as a well-defined clinicopathologic entity with a remarkable predilection for cervical lymph nodes of young women. The morphologic features encountered in lymph nodes are distinctive and should enable pathologists to establish the diagnosis with confidence. Nonetheless, this analysis of 108 cases, encountered over a 15-year period, has emphasized the difficulty that many pathologists have in recognizing this disorder, and in particular, in distinguishing it from malignant lymphoma. Although in the vast majority of cases, lymphadenopathy and other symptomatology resolves spontaneously, two of our patients, thought initially to have Kikuchi's disease, developed systemic lupus erythematosus. This raises consideration for the proposal that Kikuchi's disease may reflect a self-limited SLE-like auto-immune condition (a "forme fruste" of SLE), perhaps induced by virus-infected transformed lymphocytes. Moreover, such observations indicate that patients with Kikuchi's disease should be kept under observation for several years to ensure that they are not at risk for the development of systemic lupus erythematosus.
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            Kikuchi-Fujimoto Disease

            Abstract Kikuchi-Fujimoto disease (KFD) is a rare cause of lymphadenopathy, most often cervical. It has been mainly described in Asia. There are few data available on this disease in Europe. We conducted this retrospective, observational, multicenter study to describe KFD in France and to determine the characteristics of severe forms of the disease and forms associated with systemic lupus erythematosus (SLE). We included 91 cases of KFD, diagnosed between January 1989 and January 2011 in 13 French hospital centers (median age, 30 ± 10.4 yr; 77% female). The ethnic origins of the patients were European (33%), Afro-Caribbean (32%), North African (15.4%), and Asian (13%). Eighteen patients had a history of systemic disease, including 11 with SLE. Lymph node involvement was cervical (90%), often in the context of polyadenopathy (52%), and it was associated with hepatomegaly and splenomegaly in 14.8% of cases. Deeper sites of involvement were noted in 18% of cases. Constitutional signs consisted mainly of fever (67%), asthenia (74.4%), and weight loss (51.2%). Other manifestations included skin rash (32.9%), arthromyalgia (34.1%), 2 cases of aseptic meningitis, and 3 cases of hemophagocytic lymphohistiocytosis. Biological signs included lymphocytopenia (63.8%) and increase of acute phase reactants (56.4%). Antinuclear antibodies (ANAs) and anti-DNA antibodies were present in 45.2% and 18% of the patients sampled, respectively. Concomitant viral infection was detected in 8 patients (8.8%). Systemic corticosteroids were prescribed in 32% of cases, hydroxychloroquine in 17.6%, and intravenous immunoglobulin in 3 patients. The disease course was always favorable. Recurrence was observed in 21% of cases. In the 33 patients with ANA at diagnosis, SLE was known in 11 patients, diagnosed concomitantly in 10 cases and in the year following diagnosis in 2 cases; 6 patients did not have SLE, and 4 patients were lost to follow-up (median follow-up, 19 mo; range, 3–39 mo). The presence of weight loss, arthralgia, skin lesions, and ANA was associated with the development of SLE (p < 0.05). Male sex and lymphopenia were associated with severe forms of KFD (p < 0.05). KFD can occur in all populations, irrespective of ethnic origin. Deep forms are common. An association with SLE should be investigated. A prospective study is required to determine the risk factors for the development of SLE.
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              Kikuchi-Fujimoto's disease associated with systemic lupus erythematosus: case report and review of the literature.

              Kikuchi-Fujimoto's disease (KFD) or histiocytic necrotising lymphadenitis is a benign and self-limited disease, of unknown aetiology, which affects mainly young women. It presents with localised lymphadenopathy, predominantly in the cervical region, accompanied by fever and leukopenia in up to 50% of the cases. KFD has been rarely described in association with systemic lupus erythematosus (SLE), and its diagnosis can precede, postdate or coincide with the diagnosis of SLE. We present a patient with the diagnosis of SLE characterised by arthritis, leukopenia, malar rash, photosensitivity and positive ANA, besides cervical lymphadenopathy whose biopsy was compatible with KFD, which improved after using prednisone. Although the presence of lymphadenopathy is not uncommon in SLE patients, particularly in the phases of disease activity, the concomitance with KFD has rarely been reported in the literature. Its recognition is necessary because one can avoid laborious investigation for infectious and lymphoproliferative diseases.
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                Author and article information

                Journal
                J Investig Med High Impact Case Rep
                J Investig Med High Impact Case Rep
                HIC
                sphic
                Journal of Investigative Medicine High Impact Case Reports
                SAGE Publications (Sage CA: Los Angeles, CA )
                2324-7096
                9 June 2020
                Jan-Dec 2020
                : 8
                : 2324709620933422
                Affiliations
                [1 ]Dalin Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Dalin Town, Chiayi County
                Author notes
                [*]Chienhsiu Huang, MD, Department of Internal medicine, Division of Chest Medicine, Dalin Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, No. 2, Min-Sheng Road, Dalin Town, Chiayi County 62247. Email: hgssport@ 123456yahoo.com.tw
                Author information
                https://orcid.org/0000-0003-1239-9584
                Article
                10.1177_2324709620933422
                10.1177/2324709620933422
                7288820
                32517589
                fa22616b-2456-4bc2-b87f-9b7f20831937
                © 2020 American Federation for Medical Research

                This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License ( https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages ( https://us.sagepub.com/en-us/nam/open-access-at-sage).

                History
                : 26 January 2020
                : 19 April 2020
                : 16 May 2020
                Categories
                Case Report
                Custom metadata
                January-December 2020
                ts1

                kikuchi-fujimoto disease,pleural effusion,mediastinal lymphadenopathies

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