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      Kikuchi-Fujimoto disease associated with primary Sjögren’s syndrome – literature review based on a case report

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          Abstract

          Primary Sjögren’s syndrome (pSS) is a chronic, autoimmune disease predominantly involving exocrine glands. Lymphadenopathy is one of the possible symptoms of pSS. It may also suggest development of non-Hodgkin lymphoma (NHL), the most severe complication of pSS, or be a symptom of less common diseases, such as Kikuchi-Fujimoto disease (KFD), presented in this paper. Kikuchi-Fujimoto disease is an extremely rare, benign and self-limiting disorder, characterized by regional lymphadenopathy.

          This paper presents a case of previously unreported association of pSS, KFD and renal cancer in a patient with recurrent cervical lymphadenopathy, as well as a discussion on the coexistence of these diseases based on available literature searching for PubMed, Scopus and Google Scholar databases, particularly in this subject. These three clinical entities may manifest lymphadenopathy each, causing a diagnostic dilemma. The treatment is also challenging under such circumstances. In this particular situation, it was a combination of immunosuppressive therapy and surgery.

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          EULAR Sjogren's syndrome disease activity index: development of a consensus systemic disease activity index for primary Sjogren's syndrome.

          Raphaèle Seror, Philippe Ravaud, Simon J Bowman … (2010)
          To develop a disease activity index for patients with primary Sjögren's syndrome (SS): the European League Against Rheumatism (EULAR) Sjögren's syndrome disease activity index (ESSDAI). Thirty-nine SS experts participated in an international collaboration, promoted by EULAR, to develop the ESSDAI. Experts identified 12 organ-specific 'domains' contributing to disease activity. For each domain, features of disease activity were classified in three or four levels according to their severity. Data abstracted from 96 patients with systemic complications of primary SS were used to generate 702 realistic vignettes for which all possible systemic complications were represented. Using the 0-10 physician global assessment (PhGA) scale, each expert scored the disease activity of five patient profiles and 20 realistic vignettes. Multiple regression modelling, with PhGA used as the dependent variable, was used to estimate the weight of each domain. All 12 domains were significantly associated with disease activity in the multivariate model, domain weights ranged from 1 to 6. The ESSDAI scores varied from 2 to 47 and were significantly correlated with PhGA for both real patient profiles and realistic vignettes (r=0.61 and r=0.58, respectively, p<0.001). Compared with 57 (59.4%) of the real patient profiles, 468 (66.7%) of the realistic vignettes were considered likely or very likely to be true. The ESSDAI is a clinical index designed to measure disease activity in patients with primary SS. Once validated, such a standardised evaluation of primary SS should facilitate clinical research and be helpful as an outcome measure in clinical trials.
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            Reviewing primary Sjögren's syndrome: beyond the dryness - From pathophysiology to diagnosis and treatment

            Tim Both, Virgil Dalm, P. van Hagen … (2017)
            Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease, characterized by lymphocytic infiltration of the secretory glands. This process leads to sicca syndrome, which is the combination of dryness of the eyes, oral cavity, pharynx, larynx and/or vagina. Extraglandular manifestations may also be prevalent in patients with pSS, including cutaneous, musculoskeletal, pulmonary, renal, hematological and neurological involvement. The pathogenesis of pSS is currently not well understood, but increased activation of B cells followed by immune complex formation and autoantibody production are thought to play important roles. pSS is diagnosed using the American-European consensus group (AECG) classification criteria which include subjective symptoms and objective tests such as histopathology and serology. The treatment of pSS warrants an organ based approach, for which local treatment (teardrops, moistures) and systemic therapy (including non-steroidal anti-inflammatory drugs (NSAIDs), glucocorticoids, disease-modifying antirheumatic drugs (DMARDS) and biologicals) can be considered. Biologicals used in the treatment of pSS mainly affect the total numbers of B cells (B cell depletion (Rituximab)) or target proteins required for B cell proliferation and/or activation (e.g. B cell activating factor (BAFF)) resulting in decreased B cell activity. The aim of this review is to provide physicians a general overview concerning the pathogenesis, diagnosis and management of pSS patients.
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              The many faces of common variable immunodeficiency.

              Charlotte Cunningham-Rundles (2012)
              Common variable immunodeficiency (CVID) is a rare immune deficiency characterized by low levels of serum IgG, IgA, and/or IgM, with a loss of Ab production. The diagnosis is most commonly made in adults between the ages of 20 and 40 years, but both children and much older adults can be found to have this immune defect. The range of clinical manifestations is broad, including acute and chronic infections, inflammatory and autoimmune diseases, and an increased incidence of cancer and lymphoma. For all of these reasons, the disease phenotype is both heterogeneous and complex. In the past few years, data from large patient registries have revealed that both selected laboratory markers and clinical phenotyping may aid in separating groups of subjects into biologically relevant categories. CVID consists of 2 phenotypes, 1 in which infections are the characteristic and another in which impressive inflammatory and/or hematologic complications also develop, including lymphadenopathy, splenomegaly, autoimmune cytopenias, enteropathy, and/or and granulomatous disease. These phenotypes appear to be stable, are related to immunologic and inflammatory markers, and are predictive of outcomes. This review outlines current understanding about this syndrome based on studies of large cohorts, highlighting the evaluation and treatment of complications and, in particular, the autoimmune and inflammatory conditions that affect these patients.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Reumatologia
                Journal ID (iso-abbrev): Reumatologia
                Journal ID (publisher-id): RU
                Title: Reumatologia
                Publisher: Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie
                ISSN (Print): 0034-6233
                ISSN (Electronic): 2084-9834
                Publication date (Electronic): 31 August 2020
                Publication date (Print): 2020
                Volume: 58
                Issue: 4
                Pages: 251-256
                Affiliations
                [1 ]Department of Rheumatology and Osteoporosis, J. Struś Memorial Multidisciplinary Municipal Hospital in Poznań, Poland
                [2 ]Department of Rheumatology and Rehabilitation, Poznań University of Medical Sciences, Poland
                Author notes
                Address for correspondence: Katarzyna Wiśniewska, Department of Rheumatology and Osteoporosis, J. Struś Memorial Multidisciplinary Municipal Hospital,3 Szwajcarska St., 61-285 Poznań, Poland, e-mail: wisniewska.aisak@ 123456gmail.com
                Article
                Publisher ID: 41656
                DOI: 10.5114/reum.2020.98438
                PMC ID: 7477469
                SO-VID: cd1c2038-148b-42c8-8016-2470645158c4
                Copyright © Copyright © 2020 Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie
                License:

                This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0). License ( http://creativecommons.org/licenses/by-nc-sa/4.0/)

                History
                Date received : 24 February 2020
                Date accepted : 04 August 2020
                Categories
                Subject: Case-Based Review

                Keywords: renal cancer,lymphadenopathy,primary sjögren’s syndrome,kikuchi-fujimoto disease
                Data availability:
                Keywords: renal cancer, lymphadenopathy, primary sjögren’s syndrome, kikuchi-fujimoto disease

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