Systemic Sclerosis and Primary Biliary Cholangitis: A Comprehensive Review of Two Overlapping Rare Entities With Insights on Diagnostics and Management DOI Open Access

Hansa Thakkar,

N T Mathew,

E Prabhakar Reddy

и другие.

Cureus, Год журнала: 2025, Номер unknown

Опубликована: Апрель 10, 2025

Primary biliary cholangitis (PBC) is an autoimmune liver disease of a chronic nature that can lead to cirrhosis, predominantly in females. PBC frequently coexists with other diseases, such as systemic sclerosis (SSc), rheumatoid arthritis, lupus erythematosus, and Sjögren's syndrome. Despite variations the literature, most studies have reported few patients SSc, especially limited cutaneous subtype. Pathology SSc includes microvascular affection widespread fibrotic changes along process. This narrative review aims provide comprehensive overview existing literature up December 2024 regarding PBC, overlap syndrome emphasis on diagnostic points. Clinical manifestations be significantly overlapping for both conditions. Thus, laboratory histopathological investigations are necessary. The antibody profile cornerstone diseases. While antimitochondrial (AMA) considered specific presence anticentromere (ACA) highly suggests concomitant SSc. Several common pathologic mechanisms triggers been suggested genes like HLA-DRB1, DQA1, STAT4, IRF5 shared between two It noteworthy prognosis outcome cases affected by SSc; instance, high liver-related mortality decreases although at risk non-liver-related mortality. comprise clinical challenge diagnosis tailored management, some promising medications being investigated conditions, possibly due pathogenic mechanisms. Herein, we comprehensively available PBC-SSc terms epidemiology, underlying pathophysiology, aspects.

Язык: Английский

Systemic Sclerosis and Primary Biliary Cholangitis: A Comprehensive Review of Two Overlapping Rare Entities With Insights on Diagnostics and Management DOI Open Access

Hansa Thakkar,

N T Mathew,

E Prabhakar Reddy

и другие.

Cureus, Год журнала: 2025, Номер unknown

Опубликована: Апрель 10, 2025

Primary biliary cholangitis (PBC) is an autoimmune liver disease of a chronic nature that can lead to cirrhosis, predominantly in females. PBC frequently coexists with other diseases, such as systemic sclerosis (SSc), rheumatoid arthritis, lupus erythematosus, and Sjögren's syndrome. Despite variations the literature, most studies have reported few patients SSc, especially limited cutaneous subtype. Pathology SSc includes microvascular affection widespread fibrotic changes along process. This narrative review aims provide comprehensive overview existing literature up December 2024 regarding PBC, overlap syndrome emphasis on diagnostic points. Clinical manifestations be significantly overlapping for both conditions. Thus, laboratory histopathological investigations are necessary. The antibody profile cornerstone diseases. While antimitochondrial (AMA) considered specific presence anticentromere (ACA) highly suggests concomitant SSc. Several common pathologic mechanisms triggers been suggested genes like HLA-DRB1, DQA1, STAT4, IRF5 shared between two It noteworthy prognosis outcome cases affected by SSc; instance, high liver-related mortality decreases although at risk non-liver-related mortality. comprise clinical challenge diagnosis tailored management, some promising medications being investigated conditions, possibly due pathogenic mechanisms. Herein, we comprehensively available PBC-SSc terms epidemiology, underlying pathophysiology, aspects.

Язык: Английский

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