暂无摘要。

OBJECTIVE: Many studies reported the prevalence of extrahepatic conditions (EHC) of primary biliary cholangitis (PBC), but the great heterogeneity existed across different studies. Therefore, we conducted the systematic review and meta-analyses to determine EHC prevalence and association with PBC.
METHODS: We searched PUBMED and included observational, cross-sectional and case-controlled studies. A random or fixed effects model was used to estimate the pooled prevalence and odd ratio (OR) as appropriate.
RESULTS: Of 5370 identified publications, 129 publications with 133 studies met the inclusion criteria. Sjögren\'s syndrome had the highest prevalence (21.4 % vs. 3 % in non-PBC individuals), followed by Raynaud\'s syndrome (12.3 % vs. 1 %), rheumatoid arthritis-like arthritis (5 % vs. 3 %), systemic sclerosis (3.7 % vs. 0 %) and systemic lupus erythematosus (2 % vs. 0 %). The prevalence of overall thyroid diseases (11.3 %), autoimmune thyroid diseases (9.9 %), osteoporosis (21.1 %), celiac disease (1 %) and chronic bronchitis (4.6 %) was also increased among PBC patients.
CONCLUSIONS: This is the first exhaustive study on the old theme about EHC of PBC. Given increased prevalence of many EHCs in PBC patients, promptly recognizing these EHCs are of great importance for timely and precise diagnosis of PBC.

BACKGROUND: To investigate the risk factors for the development of pulmonary arterial hypertension (PAH) in patients with systemic lupus erythematosus (SLE).
METHODS: The literature related to risk factors for the development of PAH in SLE patients was searched by the computer on China national knowledge infrastructure (CNKI), PubMed, and Embase, and the literature search was limited to the period of library construction to October 2022. Two researchers independently performed literature screening and literature information extracting, including first author, publication time, case collection time, sample size, and study factors, and used the Newcastle-Ottawa Scale (NOS) to evaluate the quality of the literature. The relationship between each clinical manifestation and laboratory index and the occurrence of PAH in SLE patients was evaluated based on the ratio (OR value) and its 95% CI.
RESULTS: A total of 24 publications were included, including 23 case-control studies and 1 cohort study with NOS ≥ 6, and the overall quality of the literature was high. The risk of PAH was higher in SLE patients who developed Raynaud phenomenon than in those who did not [OR = 2.39, 95% CI (1.91, 2.99), P < .05]; the risk of PAH was higher in SLE patients who were positive for anti-RNP antibodies than in those who were negative for anti-RNP antibodies [OR = 1.77, 95% CI (1.17, 3.2.65), P < .05]; the risk of PAH was higher in SLE patients with interstitial lung lesions than in those without combined interstitial lung lesions [OR = 3.28, 95% CI (2.37, 4.53), P < .05]; the risk of PAH was higher in SLE patients with combined serositis than in those without serositis [OR = 2.28, 95% CI (1.83, 2.84), P < .05]. The risk of PAH was higher in SLE patients with combined pericardial effusion than in those without pericardial effusion [OR = 2.97, 95% CI (2.37, 3.72), P < .05]; the risk of PAH was higher in SLE patients with combined vasculitis than in those without vasculitis [OR = 1.50, 95% CI (1.08, 2.07), P < .05]; rheumatoid factor-positive SLE patients had a higher risk of PAH than those with rheumatoid factor-negative [OR = 1.66, 95% CI (1.24, 2.24), P < .05].
CONCLUSIONS: Raynaud phenomenon, vasculitis, anti-RNP antibodies, serositis, interstitial lung lesions, rheumatoid factor, and pericardial effusion are risk factors for the development of PAH in patients with SLE.

OBJECTIVE: Gepants are small molecules that antagonize calcitonin gene-related peptide (CGRP) receptors. Due to their favorable side effect profile and versatility in treating headaches acutely and preventively, gepants are preferred over triptans. We will cover the indications for the four FDA-approved gepants in adults: rimegepant, atogepant, ubrogepant, and zavegepant. This review will illustrate how gepants will continue to revolutionize the acute and preventive treatment of headaches.
RESULTS: Gepants are now available in oral tablet, dissolving tablet, and intra-nasal spray formulations. Recent studies have shown promising utility in treating the pre-headache or prodromal phase. They have favorable tolerability, no evidence for association with medication overuse, and remain a safer alternative in those who have cerebrovascular risk factors. Additional research is needed to explore occurrence of Raynaud\'s phenomenon in participants treated with gepants, as it has been associated with CGRP monoclonal antibodies, but are not extensively studied in gepants. Gepants are expected to play a significant role in the next generation of migraine treatments.

Raynaud phenomenon (RP) is a vasospastic condition of the digits that can be primary or secondary to systemic disease. Symptoms are triggered by cold or stress and can cause pain and skin color changes. The chronic ischemia may lead to necrosis, ulceration, and amputation. There are no Food and Drug Administration-approved treatments and cases refractory to pharmacologic and surgical treatments are difficult to control. Local botulinum toxin injections have been increasingly used in the treatment of Raynaud disease and have shown promising results.
To examine the outcomes, techniques, and complications of botulinum toxin use for the treatment of Raynaud disease.
The PubMed database was queried using \"botulinum toxin\" AND \"Raynaud phenomenon\" as title key words. Preferred reporting items for systematic reviews and meta-analysis criteria were used. Additional articles were selected while reviewing the references of the articles from PubMed. No time restrictions were followed. Articles of all languages were included. Articles were analyzed for study type, demographics, diagnosis/inclusion criteria, treatment methods, outcome measures, length of follow-up, results, and complications. A positive outcome was defined as subjective improvement in symptoms and/or improvement in the outcome measures. A poor outcome was defined as harm done to the patient by the injection that would not have occurred otherwise.
Forty-two clinical studies describing the use of botulinum toxin for Raynaud\'s phenomenon were found. A total of 425 patients with primary or secondary Raynaud\'s were treated, with ages ranging from 14 to 91 years. There were 342 women and 81 men, with a female-to-male ratio of 38:9. Outcomes were positive in 96.2% of patients. There were 14.2% of the studies that reported 3.5% of all patients showing no subjective improvement. A single study reported a poor outcome for 1 patient. There were 40.5% of the studies that reported complications, affecting 20.2% of all patients. The most frequently reported complication was transient hand weakness, affecting 44.2% of patients with complications and 8.9% of total patients. Weakness resolved in hours to months after injection. Pain at the injection site lasting minutes to days was reported in 40.7% of patients with complications, and 8.2% of total patients.
Botulinum toxin treatment for RP is effective. Complications are minor and self-limiting.

Raynaud\'s phenomenon (RP) is a cardinal feature of SSc and is associated with significant disease-related morbidity that impacts on quality of life. The assessment of SSc-RP is challenging. The aim of this scoping review was to evaluate the outcome domains studied and outcome measures used in clinical studies of SSc-RP.
Embase, MEDLINE, and the Cochrane Central Register of Controlled Trials were used to identify randomized control trials (RCTs), quasi-randomized studies, case-control studies, prospective and retrospective cohort studies, case series, and cross-sectional studies of adult participants with SSc-associated RP, written in English. A minimum of 25 participants for studies of imaging modalities and 40 participants for questionnaire-based studies was required for inclusion. Basic laboratory and genetic studies were excluded. No limitations were imposed based on intervention, comparator, or study setting. Study characteristics and primary and secondary target domains in each study were recorded.
58 studies (24 randomized clinical trials) were included in the final analysis. The commonest domains captured were severity of attacks (n=35), frequency of attacks (n=28), and duration of attacks (n=19). Objective assessments of digital perfusion were also commonly used in studies of SSc-RP.
The outcome domains and the associated outcomes used to assess the impact of SSc-RP in research studies are broad and have varied across studies. The results of this study will inform the OMERACT Vascular Disease in Systemic Sclerosis Working Group to establish a core set of disease domains encompassing the impact of RP in SSc.

OBJECTIVE: Botulinum toxin (Btx) therapy has emerged as a potential treatment for patients with Raynaud phenomenon (RP) in recent years. This study aimed to investigate the efficacy and safety of Btx treatment for RP.
METHODS: Databases of PubMed, EMBASE, Web of Science, and the Cochrane Central Register of Controlled Trials were searched from their inception up to August 2022. Studies that reported Btx use for the treatment of RP were included. A meta-analysis was conducted for the Shortened version of the Disabilities of the Arm, Shoulder, and Hand (Quick DASH) score and visual analog scale pain score using a random-effects model.
RESULTS: Thirteen full-text studies were included. The pooled standard mean changes for the visual analog scale pain score and QuickDASH score were -3.82 (95% confidence interval, -6.62 to -1.02) and 0.83 (95% confidence interval, -1.47 to -0.19), respectively. The 2 most common complications were injection site pain and intrinsic hand weakness.
CONCLUSIONS: The effect of Btx treatment on RP is promising based on current evidence. Nevertheless, more studies and randomized clinical trials with larger sample sizes are needed to confirm the current results.

Systemic sclerosis (SSc) is a systemic autoimmune disease characterised by generalized microangiopathy and fibrosis of skin and internal organs. The 2013 American College of Rheumatology (ACR) / European League Against Rheumatism (EULAR) criteria have contributed considerably to classifying patients with SSc in earlier stages, but they still lack sensitivity for a very early stage of the disease. Criteria for a very early diagnosis of SSc (VEDOSS) have been proposed by EULAR Scleroderma Trial and Research group (EUSTAR) which include three red flags: Raynaud\'s phenomenon, puffy fingers and antinuclear antibody positivity, plus SSc specific antibodies positivity and/or abnormal nailfold capillaroscopy. We report a case of a 54-year-old female patient with 6-week history of puffy fingers, Raynaud phenomenon and positive antinuclear antibodies. Further workup revealed early pathologic capillary pattern by nailfold capillaroscopy and positive anticentromere antibodies. Screening for internal organ involvement detected no heart, lung, or upper gastrointestinal tract involvement. The patient was started on pentoxifylline with further follow-up. The aim of the implementation of VEDOSS criteria is to diagnose SSc at the earliest possible stage, so that subclinical internal organ involvement could be detected and appropriate treatment started at a potentially reversible stage.

This review article summarizes the basic principles of vascular anatomy, physiology, diagnostic work-up and treatment for patients with nontraumatic upper extremity vascular disorders. Vascular disorders can be considered vasospastic or occlusive. The most commonly encountered vasospastic condition is Raynaud\'s Phenomenon secondary to scleroderma. While historically this has been managed medically with vasodilators, more advanced cases can benefit from surgical treatment to improve blood flow and minimize tissue loss, with compelling evidence that earlier surgical intervention can modify disease process and should be considered. Occlusive disease can present as aneurysm or thrombosis and often requires surgical treatment with resection of the occluded segment with or without vascular reconstruction. In advanced atherosclerotic disease or end stage ischemia, arterialization of the venous system can be considered to avoid more proximal amputations.

A rare overlap syndrome between CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome and primary biliary cholangitis (PBC) is described as PACK syndrome, an acronym for primary biliary cholangitis, anticentromere antibodies, CREST syndrome, and keratoconjunctivitis sicca. In this retrospective cohort analysis and review, we present fourteen patients who meet diagnostic criteria for PACK syndrome in one of the largest case series of this group. All patients were female, 86% of whom were White with an average age of 66.7 years (range 39-78 years). The prevalence was 5.08% in our PBC cohort (n=256) similar to previous findings. CREST syndrome was diagnosed prior to PBC in 58% of our patients and limited pulmonary and renal involvement were observed. This syndrome is rare, but given its insidious development, clinicians should be aware of this potential overlap in CREST-only and PBC-only patients.