Autoimmune liver diseases (AILDs) often coexist with other extrahepatic autoimmune diseases (EHAIDs). The spectrum of EHAIDs in patients with AILDs is similar, whereas the incidence is different. Notably, autoimmune thyroid disease and Sjogren's syndrome are the most common EHAIDs. Associated extrahepatic diseases may predate the appearance of AILDs or coincide with their onset. More frequently, they may appear during the course and even occur years after the diagnosis of AILDs. Importantly, associated EHAIDs may influence the natural course and prognosis of AILDs. To date, a definite pathophysiological pathway which contributes to the coexistence of AILDs and EHAIDs is still lacking. The current view of autoimmunity clustering involves a common susceptibility genetic background which applies to related pathologies. Herein, we review the current published researches regarding EHAIDs in patients with AILDs, particularly in relation to their clinical impact and pathophysiology. In managing patients with AILDs, gastroenterologists should be aware of the possibly associated EHAIDs to ensure a prompt diagnosis and better outcome.

[F]fluorodeoxyglucose positron emission tomography/computed tomography ([F]FDG-PET/CT) is useful for diagnosing cancers and inflammatory diseases. A polymyositis/dermatomyositis (PM/DM) lesion is an inflammatory heterogeneous disease of the striated muscle. In the present study, the maximum standardized uptake value (SUV) was compared between 22 cases with definite or probable PM/DM (PM/DM group) that underwent [F]FDG-PET/CT examination and the same number of patients with no myopathy. The average proximal muscle FDG uptake value (SUV) for each patient was represented by calculating the average of the SUV for these muscles bilaterally. The correlation between creatine kinase (CK), serum creatine kinase isoenzyme, myodynamia of the proximal limb girdle muscle and SUV of each muscle group were analyzed. The results indicated that the SUV was markedly different between the PM/DM group and the non-myopathy group. It was demonstrated that [F]FDG-PET/CT has a diagnostic value for PM/DM. The serum CK levels and the SUV were negatively correlated with myodynamia. [F]FDG-PET/CT may be used for examination to assess the severity of myositis. Furthermore, it may provide detection sites for muscle biopsy in patients with myositis.

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Aim This study aimed to retrospectively describe 15 new primary Sjögren's syndrome‐pulmonary arterial hypertension (pSS‐PAH) cases confirmed by right heart catheterization (RHC). Demographic and clinical characteristics were analyzed and risk factors for PAH in pSS were explored. Method We retrospectively described 15 new pSS‐PAH cases confirmed by RHC referred to our institution between January 2013 and March 2018. We present PAH and pSS characteristics, hemodynamic evaluations, medical management, and outcomes. A matched case control study was carried out to determine the risk factors of PAH in pSS compared with pSS‐non‐PAH patients. Results All patients were female with a mean age at PAH diagnosis of 52.9 ± 14.6 years. The delay between the first symptom and PAH diagnosis was 18.7 ± 19.7 months. The most common primary manifestation at PAH onset was exertional dyspnea (13/15). At diagnosis of PAH, PAH was severe with a mean pulmonary artery pressure of 48.8 ± 13.7 mm Hg (range, 27‐72 mm Hg) and a mean cardiac index of 2.3 ± 0.6 L/min/m2 (range, 1.47‐3.41 L/min/m2). Compared with the pSS‐PAH without pericardial effusion, pSS‐PAH with pericardial effusion had larger right arterial (53 [45‐56.75] vs 38 [35.5‐46.5], P = .018) and right ventricular sizes (47 [42.75‐51.25] vs 36 [32.5‐41], P = .007). Compared with the pSS non‐PAH group, we identified 2 risk factors for PAH in pSS: pericardial effusion (odds ratio [OR] [95% CI], 14.29 [1.14‐166.67], P = .039) and liver involvement (OR [95% CI], 14.71 [1.14‐166.67], P = .035). Conclusion For pSS patients, PAH can be the first manifestation. We believe that systemic evaluation, especially in patients with pericardial effusion and liver involvement, is important to identify high‐risk patients for PAH, improving their prognosis.

Relapsing polychondritis (RPC) is a systemic immune-mediated disease characterized by recurrent and progressive inflammation of cartilaginous tissues. 64% of RPC patients concurrent with other autoimmune disorders, there are very few reports about the concomitant RPC patients with systemic sclerosis (SSc). Herein we report a case of RPC in a 50-year-old female following SSc with pulmonary arterial hypertension (PAH) 2 years ago. She was treated with corticosteroids, immunosuppressive drugs, oral endothelin-A receptor antagonist and phosphodiesterase type 5 inhibitors. Her ocular and auricular symptoms disappeared quickly. The hemodynamic parameters were also significantly improved after treatment. To our knowledge, this is the first RPC complicated with SSc-PAH reported.