Successful Rituximab Therapy for Skin Sclerosis and Myositis in a Patient With Systemic Sclerosis, Myositis and Sjögren’s Syndrome Associated With Autoimmune Polyendocrine Syndrome Type 2 DOI Open Access
Takako Saeki, Hiroki Nishiyama, Haruna Kimura

et al.

Cureus, Journal Year: 2023, Volume and Issue: unknown

Published: Sept. 23, 2023

Autoimmune polyendocrine (or polyglandular) syndrome (APS) is a relatively rare clinical condition characterized by functional impairment of multiple endocrine glands due to loss immune tolerance. APS broadly categorized as monogenic forms, such autoimmune type 1 (APS-1), and more common polygenic variety, 2 (APS-2). Although many conditions including rheumatic diseases can develop in APS-2, systemic sclerosis or myositis complication quite no treatment strategy has yet been established. A 25-year-old man who had diagnosed having diabetes developed finger stiffness. the subjective symptoms were mild, extensive examinations various autoantibodies, hormones biopsy skin minor salivary revealed that he APS-2 (type thyroid disease) accompanied sclerosis, Sjögren’s syndrome. Rituximab therapy was initiated for progressive this resulted significant alleviation both myositis. In APS, early diagnosis immunomodulatory may arrest process before irreversible organ damage occurred. This case report suggests rituximab be promising associated with APS-2.

Language: Английский

Immunotherapy Strategy for Systemic Autoimmune Diseases: Betting on CAR-T Cells and Antibodies DOI Creative Commons
Vitaly Chasov,

Ekaterina Zmievskaya,

Irina Ganeeva

et al.

Antibodies, Journal Year: 2024, Volume and Issue: 13(1), P. 10 - 10

Published: Feb. 1, 2024

Systemic autoimmune diseases (SAIDs), such as systemic lupus erythematosus (SLE), sclerosis (SSc) and rheumatoid arthritis (RA), are fully related to the unregulated innate adaptive immune systems involved in their pathogenesis. They have similar pathogenic characteristics, including interferon signature, loss of tolerance self-nuclear antigens, enhanced tissue damage like necrosis fibrosis. Glucocorticoids immunosuppressants, which limited specificity prone tolerance, used first-line therapy. A plethora novel immunotherapies been developed, monoclonal bispecific antibodies, other biological agents target cellular soluble factors disease pathogenesis, B cells, co-stimulatory molecules, cytokines or receptors, signaling molecules. Many these shown encouraging results clinical trials. CAR-T cell therapy is considered most promising technique for curing diseases, with recent successes treatment SLE SSc. Here, we overview therapeutic approaches based on cells antibodies targeting diseases.

Language: Английский

Citations

18
The B-cells paradigm in Systemic Sclerosis: an update on pathophysiology and B-cell targeted therapies DOI Creative Commons
Cristina Scaletti, Sara Pratesi, Silvia Bellando-Randone

et al.

Clinical & Experimental Immunology, Journal Year: 2024, Volume and Issue: unknown

Published: Nov. 5, 2024

Abstract Systemic sclerosis (SSc) is considered a rare autoimmune disease in which there are alterations of both the innate and adaptive immune response resulting production autoantibodies. Abnormalities system compromise normal function blood vessels leading to vasculopathy manifested by Raynaud’s phenomenon, an early sign SSc . As consequence this reactive picture, can evolve tissue fibrosis. Several SSc-specific autoantibodies currently known associated with specific clinical manifestations prognosis. Although pathogenetic role these still unclear, their B cells plasma suggests importance development SSc. This review narratively examines B-cell dysfunctions pathogenesis discusses B-cell-targeted therapies used or potentially useful for management end-organ complications.

Language: Английский

Citations

2
Recent Advances in Treatment of Systemic Sclerosis and Morphea DOI
Noelle Teske, Nicole Fett

American Journal of Clinical Dermatology, Journal Year: 2023, Volume and Issue: 25(2), P. 213 - 226

Published: Dec. 12, 2023

Language: Английский

Citations

5
Successful rituximab therapy for skin sclerosis and myositis in a patient with systemic sclerosis, myositis and Sjögren’s syndrome associated with autoimmune polyglandular syndrome DOI Creative Commons
Takako Saeki, Hiroki Nishiyama, Shunsuke Sakai

et al.

Research Square (Research Square), Journal Year: 2023, Volume and Issue: unknown

Published: Aug. 8, 2023

Abstract Background: Autoimmune polyendocrine syndromes (APSs) are relatively rare clinical conditions characterized by functional impairment of multiple endocrine glands due to loss immune tolerance. These broadly categorized as monogenic forms, such APS-1, and a more common polygenic variety, APS-2. In APS-2, many autoimmune can develop, including rheumatic diseases. However, systemic sclerosis myositis occur quite rarecomplications, for which no treatment strategy has yet been established. Case presentation: A 25-year-old man who had diagnosed having type 1 diabetes developed finger stiffness. Although the subjective symptoms were mild, extensive examinations various autoantibodies, hormones biopsy skin minor salivary revealed that he APS-2 (type thyroid disease) accompanied sclerosis, Sjögren’s syndrome. Rituximab therapy was initiated progressive this resulted in significant both myositis. Conclusion: Various diseases develop Early diagnosis immunomodulatory may arrest process before irreversible organ damage occurred, rituximab appears be promising associated with

Language: Английский

Citations

0
Successful Rituximab Therapy for Skin Sclerosis and Myositis in a Patient With Systemic Sclerosis, Myositis and Sjögren’s Syndrome Associated With Autoimmune Polyendocrine Syndrome Type 2 DOI Open Access
Takako Saeki, Hiroki Nishiyama, Haruna Kimura

et al.

Cureus, Journal Year: 2023, Volume and Issue: unknown

Published: Sept. 23, 2023

Autoimmune polyendocrine (or polyglandular) syndrome (APS) is a relatively rare clinical condition characterized by functional impairment of multiple endocrine glands due to loss immune tolerance. APS broadly categorized as monogenic forms, such autoimmune type 1 (APS-1), and more common polygenic variety, 2 (APS-2). Although many conditions including rheumatic diseases can develop in APS-2, systemic sclerosis or myositis complication quite no treatment strategy has yet been established. A 25-year-old man who had diagnosed having diabetes developed finger stiffness. the subjective symptoms were mild, extensive examinations various autoantibodies, hormones biopsy skin minor salivary revealed that he APS-2 (type thyroid disease) accompanied sclerosis, Sjögren’s syndrome. Rituximab therapy was initiated for progressive this resulted significant alleviation both myositis. In APS, early diagnosis immunomodulatory may arrest process before irreversible organ damage occurred. This case report suggests rituximab be promising associated with APS-2.

Language: Английский

Citations

0