Concurrent Autoimmune Neutropenia and Idiopathic Thrombocytopenic Purpura Associated with IgG4-related Diease

Shimazu, Yayoi; Uchiyama, Tomoki; Mizumoto, Chisaki; Takeoka, Tomoharu; Tsuji, Masaaki; Tomo, Kenjiro; Takaori, Koji; Sakai, Naoki; Okuno, Tomoko; Ohno, Tatsuharu

doi:10.2169/internalmedicine.0190-17

Cited by 4 publications

(3 citation statements)

References 21 publications

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“…The prevalence of IgG4-RD complicated with ITP is high ( 24 - 32 ). Murase et al reported that IgG4 anti-platelet antibodies recognized a common specific antigen expressed in the pancreas and platelets ( 27 ), and Helicobacter pylori infection may cause AIP ( 27 , 33 , 34 ).…”

Section: Discussionmentioning

confidence: 99%

Autoimmune Polyglandular Syndrome Type 3 Complicated with IgG4-related Disease

et al. 2024

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A 52-year-old Japanese woman developed type 1 diabetes mellitus (type 1 DM) at 41 years old. She became complicated with Hashimoto's disease and showed swelling of both submandibular glands, which was diagnosed as IgG4-related disease (IgG4-RD). This is a rare case of a Japanese patient with autoimmune polyglandular syndrome type 3A (APS-3A) coexisting with autoimmune thyroid disease (AITD) and type 1 DM complicated by IgG4-RD. Bilateral submandibular gland resection was successfully performed without steroid therapy. We discuss the possibility that the immunological pathogenic mechanisms of APS-3A and IgG4-RD are related.

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Section: Discussionmentioning

confidence: 99%

Autoimmune Polyglandular Syndrome Type 3 Complicated with IgG4-related Disease

et al. 2024

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“…Neutrophils have been found to line bone marrow megakaryocytes in ITP, and megakaryocytes destroy platelets and inhibit platelet production [2]. Autoimmune neutropenia is associated with the occurrence of ITP and its complications [19]. In the enrichment results of the KEGG signaling pathway, we identified the MAPK signaling pathway [20], FOXO signaling pathway [21], and cytokine-cytokine receptor interaction [22] as potential therapeutic targets for ITP.…”

Section: Discussionmentioning

confidence: 99%

The Role of Follicular Regulatory T Cells/Follicular Helper T Cells in Primary Immune Thrombocytopenia

Sun

Wang

et al. 2023

Acta Haematol

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Introduction Immune thrombocytopenia (ITP) is an autoimmune disease characterized by thrombocytopenia. Herein, we sought to identify potential immune-related therapeutic targets in ITP. Methods The differentially expressed genes (DEGs) between ITP patients and controls in GSE43177 and PRJNA299534 were analyzed. The intersections of the two DEG groups were screened as common genes, and enrichment analysis was performed. Additionally, differential analysis of immune cell levels between ITP and controls was performed. Changes in the proportions of T follicular helper (Tfh) and follicular regulatory T (Tfr) cells in peripheral blood samples from ITP patients, ITP patients responding to therapy, and healthy controls were identified. The expression changes in B-cell lymphoma (Bcl)-6 and interleukin (IL)-21 were further evaluated. Results A total of 76 common genes were identified, and enrichment analysis found that these genes were mainly associated with neutrophil-mediated immunity, the MAPK signaling pathway, and the FOXO signaling pathway. Furthermore, we found different levels of Tfh cells in patients with ITP and controls. The level of Tfh cells in the peripheral blood is significantly increased in ITP patients and declines after responding to therapy. The Tfr/Tfh ratio was reduced in ITP patients and increased after responding to therapy. IL-21 and Bcl-6 were more highly expressed in ITP patients than in controls. Conclusion We identified abnormally expressed genes in ITP related to immune-related biological functions. We further identified the changes in Tfh and Tfr cells during ITP treatment. This provides a rationale for immunotherapy in ITP patients.

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“…One reason for thrombocytopenia might be the presence of anti-PLT antibody. 10,14 or IgG subclass (IgG1/3/4) of PLT antibody 3,13,[15][16][17] or autoantibody against a disintegrin and metalloproteinase with thrombospondin motifs 13 18,19 Another reason is the H. pylori infection that could lead to a decrease in PLT count. 10,16 In addition, impaired immunity due to autologous transplantation might also account for autoimmune thrombocytopenia.…”

Section: Discussionmentioning

confidence: 99%

Mikulicz’s Disease With Immune Thrombocytopenia: A New Immunoglobulin G4-Mediated Disorder and Literature Review

Cong

Chen

et al. 2019

Arch Rheumatol

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Immunoglobulin G4 (IgG4)-related Mikulicz's disease (MD) is an immune-mediated fibroinflammatory condition of unknown etiology characterized by the swelling of multiple exocrine glands including lacrimal, salivary, and parotid glands. 1,2 Immune thrombocytopenia is common in many autoimmune diseases. However, IgG4-related MD is rarely associated with immune thrombocytopenia and its pathogenic mechanism remains largely unclear. To our knowledge, only one case has been reported to date in the literature. In that case, the patient developed severe thrombocytopenia after autologous peripheral blood stem cell transplantation (PBSCT) for multiple myeloma (MM). 3 This novel study aims to report the case of a 48-year-old male patient suffering from MD with severe immune thrombocytopenia without hematological malignancies and review all reported cases of IgG4-related disease (IgG4-RD) with thrombocytopenia. CASE REPORT A 48-year-old male patient was admitted to our department with enlarged lacrimal and parotid glands associated with thrombocytopenia. He underwent resection for bilateral swelling of submandibular glands three years ago. The pathological examination revealed no tumor cells or focal lymphocytic infiltration. However, he repeatedly had enlarged lacrimal and parotid glands and dry mouth, without dry eye or arthralgia. He was then diagnosed with Sjögren syndrome (SS) and received anti-rheumatic treatment with hydroxychloroquine and Tripterygium wilfordii (a traditional Chinese medicine having definite effects on autoimmune and inflammatory diseases) for two years. However, the patient suffered from repeated recurrences of enlarged glands and presented with thrombocytopenia after two years. Physical examination revealed nontender bilateral swelling of lacrimal and ABSTRACT Mikulicz's disease (MD), an immunoglobulin G4 (IgG4)-related disease, is a fibrosis-associated inflammatory disease characterized by painless bilateral swelling of lacrimal and salivary glands. Although thrombocytopenia is associated with many rheumatologic disorders, it is rare in IgG4-related MD. This novel study aims to report a rare case of IgG4-related MD complicated with severe autoimmune thrombocytopenia without any hematological malignancies and review all cases of IgG4-related disease associated with thrombocytopenia in the literature.

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Concurrent Autoimmune Neutropenia and Idiopathic Thrombocytopenic Purpura Associated with IgG4-related Diease

Cited by 4 publications

References 21 publications

Autoimmune Polyglandular Syndrome Type 3 Complicated with IgG4-related Disease

Autoimmune Polyglandular Syndrome Type 3 Complicated with IgG4-related Disease

The Role of Follicular Regulatory T Cells/Follicular Helper T Cells in Primary Immune Thrombocytopenia

Mikulicz’s Disease With Immune Thrombocytopenia: A New Immunoglobulin G4-Mediated Disorder and Literature Review

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