Hepatobiliary involvement in systemic sclerosis and the cutaneous subsets: Characteristics and survival of patients from the Spanish RESCLE Registry

Marí-Alfonso, Begoña; Simeón-Aznar, Carmen Pilar; Guillén‐Del‐Castillo, Alfredo; Rubio‐Rivas, Manuel; Trapiella-Martínez, Luis; Todolí-Parra, José Antonio; Carballeira, Mónica Rodríguez; Marín-Ballvé, Adela; Iniesta-Arandia, Nerea; Colunga-Argüelles, Dolores; Castillo-Palma, María Jesús; Sáez‐Comet, Luis; Egurbide-Arberas, María Victoria; Ortego-Centeno, Norbérto; Freire, Mayka; Hitos, José Antonio Vargas; Chamorro, Antonio-Javier; Madroñero-Vuelta, Ana Belén; Perales-Fraile, Isabel; Pla-Salas, Xavier; Fernández-De-La-Puebla, rafael A.; Fonollosa-Plà, Vicent; Tolosa-Vilella, Carles

doi:10.1016/j.semarthrit.2017.10.004

Cited by 23 publications

(16 citation statements)

References 51 publications

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“…In the literature, all three previous cases of PBC/AIH OS and SSc were in the limited cutaneous form [1][2][3] . Based on these findings, some authors have suggested that the lcSSc subset may have a distinct pathogenesis with a greater autoimmune background 7 . However, our patient's condition shows that dcSSc can also coexist with PBC/AIH OS and that a common autoimmune basis may exist between AILDs and SSc-irrespective of the SSc cutaneous subtype.…”

Section: Discussionmentioning

confidence: 97%

“…Historically, hepatobiliary involvement in SSc has not been considered characteristic; however, recent investigations have revealed a higher prevalence of liver disturbances in SSc patients ranging from 37% to 52% 7 . Interestingly, primary AILDs accounted for 77% of all liver diseases in SSc patients with PBC as the main cause (57%) followed by AIH (16%) 7 . Previously, some researchers argued that hepatobiliary involvement in SSc occurs through chance and is possibly related to coexisting liver steatosis, viral infections, or drug-induced toxicity from SSc treatment 8 .…”

Section: Discussionmentioning

confidence: 99%

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Diffuse Systemic Sclerosis in a Patient with Primary Biliary Cirrhosis and Autoimmune Hepatitis Overlap Syndrome: A Case Report

et al. 2020

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Systemic sclerosis (SSc) is a chronic systemic disease of unknown etiology characterized by vasculopathy, excessive accumulation of extracellular matrix, and fibrosis of the skin and other internal organs. Although its etiology remains elusive, approximately one third of SSc patients presents with additional autoimmune disease, which suggests that an autoimmune mechanism is a major component of the underlying pathophysiology. On the other hand, primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) are two main autoimmune liver diseases. A 41-year-old female previously diagnosed with PBC/AIH overlap syndrome presented with multiple, painful brownish to erythematous firm patches on the hands, arms, axillae, neck, abdomen, and thighs. Laboratory work-up yielded positive results for anti-nuclear antibody, anti-Ro/Sjögren's-syndrome-related antigen A autoantibodies, and perinuclear anti-neutrophil cytoplasmic antibodies while punch biopsy of her left hand showed characteristics that are consistent with scleroderma. Herein, we report the first case of a patient with diffuse cutaneous SSc and concurrent PBC/AIH overlap syndrome and suggest that this coexistence of multiple autoimmune diseases is not a coincidence but rather that a common autoimmune pathogenesis may exist.

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Section: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 99%

Diffuse Systemic Sclerosis in a Patient with Primary Biliary Cirrhosis and Autoimmune Hepatitis Overlap Syndrome: A Case Report

et al. 2020

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“…Furthermore, our study showed that centromere-type nuclear ANA pattern was significantly associated with the reticular cytoplasmic ANA pattern. Anti-centromere antibody is an immunological marker of systemic sclerosis, which is known to be associated with the propensity of developing AILD [27,28]. Herein, among 11 patients with positive anti-centromere nuclear pattern, one patient was diagnosed with systemic sclerosis, whereas the rest did not manifest any clinical signs of systemic sclerosis.…”

Section: Plos Onementioning

confidence: 95%

The significance of cytoplasmic antinuclear antibody patterns in autoimmune liver disease

et al. 2021

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We aimed to determine the significance of cytoplasmic antinuclear antibody (ANA) patterns using computer-aided immunofluorescence microscopy in patients with autoimmune liver diseases (AILD). ANA staining pattern was identified by treating cultured human epithelial type 2 (HEp-2) cells with the sera of the patients. Medical records of patients with suspected AILD who had positive cytoplasmic ANA patterns between February 2017 and November 2019 were retrospectively reviewed for clinical, laboratory, and immunological data. Cytoplasmic ANA patterns of AILD and non-AILD groups were compared. Further subgroup analysis of patients with AILD who had reticular or speckled cytoplasmic ANA patterns was conducted. We found that among the 196 patients with positive cytoplasmic ANA patterns, 113 (57.6%) were diagnosed with AILD. The percentage of reticular cytoplasmic pattern was higher in the AILD group than that in the non-AILD group (64.0% vs. 21.9%, p < 0.001). Furthermore, patients with AILD who exhibited a reticular ANA pattern demonstrated a higher positive rate for anti-mitochondrial antibodies (66.7% vs. 2.6%, p < 0.001) than those who exhibited the speckled ANA pattern. Moreover, AILD patients with the reticular ANA pattern displayed a lower positive rate for anti-smooth muscle antibodies (0% vs. 45%, p < 0.001) and nuclear ANA pattern (73.2% vs. 97.5%, p = 0.003) than those with the speckled ANA pattern. Therefore, cytoplasmic ANA patterns could be used to guide AILD characterization in suspected AILD cases, especially as the reticular ANA pattern is strongly associated with AILD. Thus, it is important to check cytoplasmic ANA patterns for AILD evaluation, even when nuclear ANA patterns are negative.

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“…The Spanish scleroderma registry has not only characterized the Spanish population with SSc 109 but has also made advances in the characterization of liver involvement 110 , vascular 111 , ILD 112 , and PAH 113 , as well as the role of phosphodiesterase inhibitors and endothelin receptor antagonists as bi-therapies in the prognosis of PAH versus monotherapy 114 . The preventive role of these drugs in the development of PAH and the renal crisis has also been evaluated 115 .…”

Section: Spain 2006mentioning

confidence: 99%

Nationwide and international registries on scleroderma. Past, present, and future

Rubio‐Rivas¹

2021

SJMED

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Systemic sclerosis (SSc) is a low-incidence autoimmune disease that requires collaborative work through national or international registries to advance scientifically. The present systematic review aims to be an update of all national and international registries to date. In total, 7 first-world countries have SSc registries. In addition, there are also 3 international SSc registries plus 2 other registries focused on SSc-related pulmonary arterial hypertension. These registries not only carry out clinical research but also allow the collection of serum, DNA and tissue samples, as well as collaborative work to carry out randomized clinical trials and collaboration with other registries not directly focused on SSc.

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Hepatobiliary involvement in systemic sclerosis and the cutaneous subsets: Characteristics and survival of patients from the Spanish RESCLE Registry

Cited by 23 publications

References 51 publications

Diffuse Systemic Sclerosis in a Patient with Primary Biliary Cirrhosis and Autoimmune Hepatitis Overlap Syndrome: A Case Report

Diffuse Systemic Sclerosis in a Patient with Primary Biliary Cirrhosis and Autoimmune Hepatitis Overlap Syndrome: A Case Report

The significance of cytoplasmic antinuclear antibody patterns in autoimmune liver disease

Nationwide and international registries on scleroderma. Past, present, and future

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