Janus kinase inhibition (JAKi) therapy in refractory anti-synthetase syndrome: A retrospective cohort study.

OBJECTIVES: To evaluate the efficacy and safety of Janus kinase inhibitors (JAKi) in the treatment of refractory anti-synthetase syndrome (ASS) in real-world clinical settings. METHODS: The medical records of all refractory ASS patients who were treated with JAKi from October 2020 to June 2023 were retrospectively reviewed. RESULTS: Twenty patients were included, and all (100 %) patients had interstitial lung disease (ILD). After treatment with JAKi, 14 (70 %) of the refractory ASS patients showed significant improvement in clinical manifestations, including arthritis (56.3 % vs. 6.3 %, p = 0.002), rash (77.8 % vs. 27.8 %, p = 0.012), shortness of breath (55.6 % vs. 16.7 %, p = 0.039), cough (61.1 % vs. 11.1 %, p = 0.012). Improvement was noted for myalgia (50 % vs. 11.1 %, p = 0.016) and muscular weakness (61.1 % vs. 11.1 %, p = 0.012), while creatine kinase (CK) levels, which were abnormally elevated in five patients prior treatment, were significantly lowered (1096 ± 1042.98 IU/L vs. 199.2 ± 144.66 IU/L, p = 0.043). A decrease in levels of inflammatory markers, including erythrocyte sedimentation rate (ESR) (p = 0.001) and C-reactive protein (CRP) (p = 0.023) was observed in the patients. In ASS-ILD, the CT score reduced (188.75 ± 69.67 vs. 156.35 ± 74.62, p = 0.001). Furthermore, the glucocorticoid dose significantly reduced (21.42 ± 13.26 mg vs. 11.32 ± 8.59 mg; p = 0.001). CONCLUSIONS: JAKi were effective in most refractory ASS patients as evidenced by improved skin rash, myositis, and ILD. However, larger prospective controlled studies are required to evaluate its efficacy.

Prevalence, Risk Factors, and Mortality of Invasive Pulmonary Aspergillosis in Patients with Anti-MDA5+ Dermatomyositis: A Retrospective Study in China.

Objective: To investigate the prevalence, risk factors and prognosis of invasive pulmonary aspergillosis (IPA) in patients with anti-melanoma differentiation-associated gene 5 positive dermatomyositis (anti-MDA5+ DM). Methods: A retrospective analysis was conducted in anti-MDA5+ DM patients diagnosed between January 2016 and March 2023. Patients with lower respiratory tract specimens were categorized into IPA+ and IPA- groups based on the presence of IPA and their clinical characteristics and prognoses then compared. Results: Of the 415 patients diagnosed with anti-MDA5+ DM, 28 cases had IPA (prevalence rate of 6.7%) with Aspergillus fumigatus being the most common species. The patients were categorized into IPA+ (n=28) and IPA- (n=98) groups, with no significant age or gender-related differences (P>0.05). The IPA+ group had a lower lymphocyte count, particularly the CD4+ T-cell count, and reduced serum albumin and higher serum ferritin levels (P all<0.05). An elevated bronchoalveolar lavage fluid (BALF) galactomannan level was found to be the sole independent risk factor for the occurrence of IPA (adjusted OR=2.191, P=0.029) with a cut-off value of 0.585 and area under the curve of 0.779. The mortality rate in the IPA+ group was 25%. Compared to survivors, non-survivors in this group exhibited a higher incidence of rapidly progressive interstitial lung disease, lower lymphocyte counts, and increased co-infection with Pneumocystis jirovecii (P all<0.05). Conclusion: IPA was not rare in patients with anti-MDA5+ DM, with elevated BALF galactomannan levels being an independent risk factor for IPA occurrence. Clinicians must exercise vigilance to identify patients exhibiting the aforementioned risk factors.

Interstitial Lung Disease in Patients with Mixed Connective Tissue Disease: A Retrospective Study.

Objective: To investigate the clinical features, severity and prognosis of interstitial lung disease (ILD) in patients with mixed connective tissue disease (MCTD). Methods: We performed a retrospective study on clinical data of MCTD patients admitted to China-Japan Friendship Hospital between October 2012 and October 2022. Data including long-term follow-up were retrieved from medical records. We compared MCTD patients with and without ILD in terms of clinical features, laboratory and imaging findings, severity and treatment response. Results: A total of 59 patients were included, with a mean age of 46 years, among which 91.5% (n = 54) were females. Symptoms of pulmonary involvement were present in 44 patients (74.6%, 95% CI: 62.3-84.9%). Based on lung high-resolution computed tomography (HRCT), ILD was diagnosed in 39 (66.1%) patients, among which 31 (79.5%) showed nonspecific interstitial pneumonia (NSIP) as the radiological pattern, 21 (53.9%) showed a reticulation pattern, while 24 (61.5%) showed ground glass opacity (GGO). Eight (13.6%) patients had pulmonary arterial hypertension (PAH), and 7 (11.9%) had pleural effusions. Based on pulmonary function tests (PFTs), 27 patients were divided into the mild 13 (48.1%) and moderate 14 (51.9%) groups. Multivariate analysis showed that gastroesophageal reflux (GER; OR=5.28, p=0.010) and cough (OR=4.61, p=0.043) were the predictive factors for ILD. With a median follow-up of 50 months, the mortality rate was 2.38%. Conclusion: ILD is common in MCTD patients, with NSIP as the common imaging pattern. Patients with GER and cough are relevant factors in the development of ILD. The majority of MCTD patients with ILD are mild to moderate in severity.

Effectiveness and safety of eltrombopag in connective tissue disease patients with refractory immune thrombocytopenia: a retrospective study.

Objectives: We aimed to investigate the safety and effectiveness of eltrombopag for adult patients with refractory immune thrombocytopenia (ITP) secondary to connective tissue disease (CTD). Methods: This is a single-centre, retrospective cohort and propensity score-matched study. Data from CTD-ITP patients treated with eltrombopag between January 2019 and January 2023 were retrospectively analysed. Baseline characteristics and follow-up information were recorded. CTD patients without ITP were matched to identify the risk factors associated with CTD-ITP performed by Logistic regression analysis. Results: Twenty patients were enrolled, including 5 systemic lupus erythematosus (SLE), 9 Sjögren's syndrome (SS) and 6 undifferentiated connective tissue disease (UCTD). Nineteen (95%) patients were female, and the median age was 59 years. Logistic regression analysis showed that anaemia (OR = 8.832, P = 0.007) was associated with increased risk of ITP, while non-erosive arthritis (OR = 0.045, P = 0.001) and interstitial lung disease (OR = 0.075, P = 0.031) were associated with reduced risk. Fourteen patients (70%) achieved a complete response (CR) and one (5%) achieved a partial response (PR). The median response time was 14 days. The median platelet count was 8.5 × 109/l at baseline of eltrombopag and increased to 122 × 109/l after 4 weeks. No adverse events were observed. Conclusions: Eltrombopag appears to be effective, safe and well-tolerated in refractory ITP patients with CTD; larger studies are needed to confirm the generalizability of these findings.

Nintedanib could potentially lead to improvements in anti-melanoma differentiation-associated 5 dermatomyositis-associated interstitial lung disease.

OBJECTIVES: To determine the efficacy and safety of nintedanib in patients with anti-melanoma differentiation-associated gene 5 antibody positive dermatomyositis-associated interstitial lung disease (anti-MDA5+ DM-ILD). METHODS: The study was a retrospective cohort design that evaluated patients with anti-MDA5+ DM who either received or did not receive nintedanib. Clinical symptoms, laboratory tests, and survival were compared in the two groups using a propensity score-matched analysis. The primary endpoint was mortality, while adverse events were recorded descriptively. RESULTS: After propensity score matching, 14 patients who received nintedanib (nintedanib+ group) and matched 56 patients who did not receive nintedanib (nintedanib- group) were enrolled. Compared with the nintedanib- group, the nintedanib+ group had a lower incidence of heliotrope and arthritis, higher lymphocyte counts, lower serum ferritin levels, and greater 12-month survival (all p<0.005). Although lung function, HRCT score, and lung VAS were not statistically different between the two groups, the longitudinal study showed significant improvement in HRCT scores (p=0.028) and pulmonary VAS (p=0.019) in the nintedanib+ group. Adverse events occurred in 28.6% of patients, with the most common adverse event with nintedanib being diarrhoea. CONCLUSIONS: Nintedanib may be effective for improving clinical symptoms, laboratory parameters, lung lesions, and survival in anti-MDA5+ DM. Diarrhoea was the most common adverse event associated with nintedanib, although the drug was well tolerated by most patients.

Macrophage activation syndrome in juvenile dermatomyositis: a case report and a comprehensive review of the literature.

BACKGROUND: Macrophage activation syndrome (MAS) is a severe and life-threatening syndrome associated with autoimmune diseases. The coexistence of MAS and juvenile dermatomyositis (JDM) is not well reported. This report describes a case of JDM with MAS and summarizes the clinical characteristics and prognosis of MAS in patients with JDM. CASE PRESENTATION: The patient was a 15-year-old female with JDM, presenting with heliotrope rash, muscle weakness, increased muscle enzyme, anti-nuclear matrix protein 2 (NXP2) antibody, and muscle biopsy consistent with JDM. The patient developed fever, cytopenia, and hyperferritinemia three months after the first manifestations. Hemophagocytosis was found in the bone marrow. The final diagnosis was JDM combined with MAS. Despite intensive treatment, the patient died of MAS. By reviewing the literature, we found 17 similar cases. Together with the present case, 18 patients were identified, the median age of disease onset was 13.5 years, and male to female ratio was 1.25: 1. Nine out of 16 (56.3%) patients were complicated with interstitial lung disease (ILD). The median time interval between JDM onset and MAS diagnosis was 9 weeks. At the onset of MAS, all (100%) patients had elevated levels of ferritin and serum liver enzymes. Among 18 patients, 14 (77.8%) had fever, 14/17 (82.4%) had cytopenia, 11/11 (100%) had hepatosplenomegaly, and 13/14 (92.9%) had hemophagocytosis. Five (27.8%) patients showed central nervous system (CNS) involvement. The mortality of MAS rate of in patients with JDM was 16.7%, despite various treatment methods. CONCLUSION: . The coexistence of JDM and MAS is underestimated with increased mortality. Hepatosplenomegaly and increased serum levels of ferritin in patients with JDM should raise clinical suspicion for MAS.

Clinical characteristics of myositis patients with isolated anti-U1 ribonucleoprotein antibody resemble immune-mediated necrotizing myopathy.

Background: Anti-U1 ribonucleoprotein (U1RNP) antibodies were associated with connective tissue diseases (CTDs), but the clinical characteristics of this antibody in Chinese myositis patients have not been studied. Objective: We aim to analyze the clinical features of myositis patients who test positive for anti-U1RNP antibodies and delineate a subgroup of myositis. Design: This is a retrospective cohort study. Methods: We reviewed the clinical data of myositis patients with anti-U1RNP antibodies and compared them to those with anti-signal recognition particle (SRP) and hydroxy-3-methylglutaryl-CoA reductase (HMGCR) antibody-associated immune-mediated necrotizing myopathy (IMNM). Results: A total of 30 adult cases were identified; median age was 47.5 years and 24 (80%) were females, and 12 patients did not coexist with myositis-specific antibodies (MSAs) (isolated anti-U1RNP). The serum creatine kinase (CK) was significantly higher in patients with isolated anti-U1RNP (2182.5 U/L versus 289 U/L, p = 0.01), and the number of patients with CK > 2000 U/L was higher compared to that in anti-U1RNP antibody patients coexisting with MSAs (66.7% versus 16.7%, p = 0.009). The prevalence of IMNM in patients' muscle pathology with isolated anti-U1RNP was significantly higher (75%, p = 0.003). Skin rashes were less common in isolated anti-U1RNP group (p < 0.05). Of the 25 individuals with available pulmonary high-resolution CT (HRCT), 14 (56%) were diagnosed with interstitial lung disease (ILD). The incidence of muscular weakness, dysphagia, or levels of CK was not different between the isolated anti-U1RNP antibody individuals and the anti-HMGCR or SRP-IMNM groups (p > 0.05). But the frequency of Raynaud's phenomenon, arthritis, and membrane attack complex (MAC) deposits in myositis patients with isolated anti-U1RNP antibodies were higher than in anti-HMGCR and SRP-IMNM (all p < 0.005). There was no difference between anti-U1RNP patients with and without Ro-52 (p > 0.05). Isolated anti-U1RNP individuals showed marked improvements in muscle strength, and the remission rate in 1 and 2 years was significantly higher than that in anti-HMGCR and SRP-IMNM (p < 0.05). Conclusions: The clinical and pathological features of myositis patients with isolated anti-U1RNP antibodies were similar to IMNM. Arthritis and ILD are the most common extramuscular clinical features. Most respond well to treatment and have a good prognosis.

High prevalence and mortality of Pneumocystis jirovecii pneumonia in anti-MDA5 antibody-positive dermatomyositis.

OBJECTIVES: To identify potential risk factors and prognostic factors of Pneumocystis jirovecii pneumonia (PJP) infection in anti-melanoma differentiation-associated gene 5 antibody-positive DM (anti-MDA5+ DM) patients, and to evaluate the diagnostic performance of metagenomic next-generation sequencing (mNGS). METHODS: Anti-MDA5+ DM patients who underwent mNGS or real-time PCR for PJP detection were recruited. The potential risk factors for PJP occurrence and death were analysed via Logistic regression and Cox proportional hazards regression, respectively. The diagnostic efficacy of mNGS was compared with the conventional methods. RESULTS: 91 patients were enrolled and 44 were assigned to PJP+ group. The PJP detection rate was 48.4%. PJP often occurred in the first 3 months (68.2%) of the disease; this period also showed the highest mortality rate (20.5%). Fever and increased lactate dehydrogenase (LDH) were independent risk factors for PJP occurrence, while trimethoprim-sulfamethoxazole (TMP/SMZ) prophylaxis was an independent protective factor (all P < 0.05). Older age and increased LDH were predictors for mortality in patients with anti-MDA5+ DM and PJP (all P < 0.05). In addition, we found that mNGS had a sensitivity of 100.0% and specificity of 90.0% in diagnosing PJP, with the highest area under the curve of 0.95 (P < 0.001). CONCLUSION: PJP has high prevalence and mortality in anti-MDA5+ DM. It is crucial for clinicians to identify high-risk patients and promptly institute TMP/SMZ to prevent PJP. mNGS is the preferred approach for pathogen detection in anti-MDA5+ DM when PJP is suspected.

Inflammatory myopathy following coronavirus disease 2019 vaccination: A systematic review.

Introduction: Reports of unexpected side effects have accompanied the vaccination of larger proportions of the population against coronavirus disease 2019 (COVID-19), including a few cases of inflammatory myopathy (IM). In a bid to improve understanding of the clinical course of vaccine complications, a systematic review of reported cases of IM following COVID-19 vaccination has been conducted. Methods: The PRISMA guideline 2020 was followed. Two independent investigators systematically searched PubMed and Embase to identify relevant studies published up to July 2022, using the following keywords: COVID-19 Vaccine, inflammatory myositis. The Joanna Briggs Institute critical appraisal tools were used for the risk of bias. Results: A total of 24 articles presenting clinical features of 37 patients with IM following COVID-19 vaccine were identified. Female patients composed 59.5% of cases and 82.4% had been vaccinated with BNT162b2 or ChAdOx1. Onset of symptoms occurred within 2 weeks of the first or second vaccine dose in 29 (85.3%) patients and included muscular weakness in 54.1% and skin rash in 71.4% of patients. Myositis specific autoantibodies (MSAs) and myositis associated autoantibodies (MAAs) were reported in 28 patients. Specific clinical subtypes of myositis, reported in 27 patients, included 22 (81.5%) cases of dermatomyositis (DM) and 3 (11.1%) cases of immune-mediated necrotizing myopathy (IMNM). Following treatment, 32 (86.5%) patients showed improvement on follow-up. Conclusion: COVID-19 vaccine may induce various clinical myositis subtypes and related antibodies. Muscular weakness was the most common presenting symptom. Clinicians should be aware of this unexpected adverse event following COVID-19 vaccination and arrange for appropriate management. Systematic review registration: INPLASY https://inplasy.com/inplasy-2022-9-0084/ [INPLASY202290084].

The clinical features and prognoses of anti-MDA5 and anti-aminoacyl-tRNA synthetase antibody double-positive dermatomyositis patients.

Objective: To explore the clinical features and prognoses of dermatomyositis (DM) associated with a double-positive anti-MDA5 and anti-aminoacyl-tRNA synthetase (anti-ARS) antibody presentation. Methods: We retrospectively analyzed 1280 consecutive patients with idiopathic inflammatory myopathy (IIM). Individuals with anti-MDA5 and anti-ARS antibodies (anti-MDA5+/ARS+) were compared to anti-MDA5-/ARS+ and anti-MDA5+/ARS- control individuals based on clinical, pulmonary radiological characteristics, treatment, and follow-up information. Results: Six individuals (0.47%) presented with anti-MDA5+/ARS+; of these, 2 (33.3%) were anti-PL-12+, 2 (33.3%) were anti-Jo-1+, 1 (16.7%) was anti-EJ+, and 1 (16.7%) was anti-PL-7+. Hallmark cutaneous manifestations, including Gottron's sign (100%), heliotrope rash (50%), mechanic's hand (66.7%), and skin ulcers (16.7%) were common. Anti-MDA5+/ARS+ patients tended to have higher ferritin levels (p = 0.038) than anti-MDA5-/ARS+ group, and higher CD4+ T-cell counts (p = 0.032) compared to the anti-MDA5+/ARS- group. Radiologically, NSIP with OP overlap was predominant (60%). Consolidation (60%), ground-glass attenuation (GGA) (80%), traction bronchiectasis (80%), and intralobular reticulation (100%) were common in anti-MDA5+/ARS+ individuals. All were diagnosed with ILD and 50% were categorized as RPILD. All patients received glucocorticoids combined with one or more immunosuppressants. Most (83.3%) had a good prognosis following treatment, but there was no difference in the survival rate between the three subgroups. Conclusion: Presentation with anti-MDA5+/ARS+ DM was rare. The clinical and radiological characteristics of anti-MDA5+/ARS+ DM combined the features of anti-MDA5+ and anti-ARS+ individuals. Individuals with anti-MDA5+/ARS+ antibodies may respond well to glucocorticoid therapy; glucocorticoids combined with one or more immunosuppressants may be considered a basic treatment approach.

Anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis complicated with macrophage activation syndrome.

Background: Anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis (DM) has low survival rate, whereas macrophage activation syndrome (MAS) is a severe and life-threatening syndrome associated with autoimmune diseases. Their coexistence is very rare. This study aimed to describe the prevalence, clinical characteristics, and outcomes of anti-MDA5 antibodies-positive DM patients complicated with MAS. Methods: In this retrospective study, we enrolled DM patients with anti-MDA5 antibodies, who were hospitalized between 2016 and 2020 and included patients diagnosed with MAS. Results: We identified four (2%) DM patients with anti-MDA5 antibodies. They were females with interstitial lung disease (ILD). The level of aspartate aminotransferase (AST), lactate dehydrogenase (LDH), and ferritin were significantly higher in the MAS group than those without MAS (p < 0.05). Patients with MAS were significantly more likely to develop a dysphagia (p = 0.012). Literature review revealed eight similar cases. Together with the present study, we identified 12 patients complicated with ILD. The median age of disease onset was 52 years with a male to female ratio of 1:6. The median duration between DM onset and MAS diagnosis was 3 months. The mortality of MAS in anti-MDA5 antibody-positive DM was 50%. Patients who died were older than those who survived (56.7 years versus 35.5 years; p = 0.015). Conclusions: MAS was rare in anti-MDA5 antibody-positive DM. The higher the level of AST, LDH, and ferritin, the greater the risk of MAS. They were associated with high mortality rates, particularly in older patients.

Thromboembolic events in idiopathic inflammatory myopathy: a retrospective study in China.

OBJECTIVE: To investigate the clinical characteristics and risk factors for thromboembolic events in patients with idiopathic inflammatory myopathy (IIM). METHODS: We retrospectively analyzed 1144 consecutive patients with IIM for arterial and venous thromboses and compared them with age- and sex-matched IIM patients without thrombosis. Logistic regression analysis was used to analyze risk factors for thrombosis. RESULTS: Twenty-four (2.1%) patients had arterial or venous thromboses (mean age, 62.6 ± 11.6 years; range, 33-81 years). Thromboembolic events occurred in 54.2% (13/24) of patients within 6 months before or after IIM diagnosis. Thrombosis patients had a higher Cutaneous Dermatomyositis Disease Area and Severity Index score (p = 0.028), higher myositis disease activity assessment visual analogue scale score (MYOACT) (p < 0.001), and a greater proportion of them had varicose veins (p = 0.001), surgical history in the past 3 months (p = 0.039), malignancy (p = 0.018), and infection (p < 0.001). The manual muscle test 8 score (p < 0.001) and albumin level (p = 0.003) were lower in thrombosis patients. There was no significant difference between the two groups in glucocorticoid pulse therapy; however, intravenous immunoglobulin therapy was more commonly used in thrombosis patients (p = 0.04). In multivariable regression models, malignancy, infection, longer duration of glucocorticoid treatment, and higher MYOACT were risk factors for thrombosis. The cumulative survival time of IIM patients with thrombosis was significantly shorter than that of controls. CONCLUSIONS: Malignancy, infection, longer duration of glucocorticoid use, and increased myositis disease activity are risk factors for thrombosis. Patients with these risk factors should undergo screening for thrombosis. Key Points • To investigate the clinical characteristics and risk factors for thromboembolism events in patients with IIM, we performed a retrospective study with IIM patients who experienced a thromboembolic event. • We found that malignancy, infection, longer duration of glucocorticoid treatment, and a higher level of myositis disease activity were risk factors for thrombosis. • The results suggest that patients with the above risk factors should undergo screening for thrombosis.

The Clinical Phenotype of Chinese Patients With Autoimmune Pancreatitis Differs Significantly From Western Patients.

Aim: To characterize the clinical features of autoimmune pancreatitis (AIP) in China and compare differences between our Chinese cohort and Western cohorts. Methods: This was a retrospective study of patients with AIP that was carried out in the China-Japan Friendship Hospital between January 2010 and April 2021. We included a total of 50 patients (46 males and 4 females) aged between 27 and 86 years who fulfilled the international Consensus Diagnostic (ICD) Criteria. For comparative purposes, we included data from seven representative Western cohorts. Result: When comparing Chinese and Western patients, we found that obstructive jaundice was the most frequent initial symptom (68 vs. 43%, P < 0.001). Extra-pancreatic organ involvement was more common in Chinese patients (68 vs. 30%, P < 0.001). Sclerosing cholangitis was the most frequent extrapancreatic lesion (48 vs. 24%, P = 0.001). The elevation of serum IgG4 was more obvious in our cohort (86 vs. 49%, P < 0.001). Conversely, the rates of ANA-positivity were significantly higher in Western populations (17 vs. 50%, P = 0.006). With regards to imaging, diffuse swelling was significantly more common in China (44 vs. 27%, P = 0.021). Steroid therapy was used more frequently in our Chinese patients (84 vs. 59%, P = 0.001). The steroid-response rate was also significantly higher in our Chinese patients (85 vs. 54%, P = 0.001); However, the rate of resection was higher in Western cohorts (2 vs. 31%, P < 0.001). There was no significant difference between the two populations with regards to recurrence rate (33 vs. 33%, P = 1.000). Conclusion: This study identified significant differences between Chinese and Western populations of patients with AIP. Within the Chinese population, AIP was more likely to have jaundice and extra-pancreatic organ involvement, and elevated serum IgG4 levels. Chinese patients were also showed favorable responses to treatment with glucocorticoids.

Interstitial lung disease is not rare in immune-mediated necrotizing myopathy with anti-signal recognition particle antibodies.

OBJECTIVES: The purpose was to clarify the characteristics of interstitial lung disease (ILD) in immune-mediated necrotizing myopathy (IMNM) patients with anti-signal recognition particle (SRP) antibodies. METHODS: Medical records of IMNM patients with anti-SRP antibodies were reviewed retrospectively. RESULTS: A total of 60 patients were identified. Twenty-seven (45.0%) patients were diagnosed with ILD based on lung imaging: nonspecific interstitial pneumonia (NSIP) in 17 patients (63.0%) and organizing pneumonia in 9 patients (33.3%). Reticulation pattern was identified in 17 patients (63.0%) whereas 10 cases (37.0%) showed ground glass opacity and patchy shadows by high-resolution computed tomography (HRCT). Pulmonary function tests (PFTs) were available in 18 patients, 6 (33.3%) and 10 (55.6%) patients were included in the mild and moderate group, respectively. The average age at the time of ILD onset was significantly older than those without ILD (48.6 ± 14.4 years vs. 41.2 ± 15.4 years, p < 0.05), and the frequency of dysphagia in the ILD group was higher than the group without ILD (p < 0.05). Long-term follow-up was available on 9 patients. PFTs were stable in 8 (88.9%), and the HRCT remained stable in 6 (66.7%) patients. CONCLUSIONS: ILD is not rare in IMNM patients with anti-SRP antibodies, most being characterized as mild to moderate in severity. NSIP is the principal radiologic pattern, and ILD typically remains stable following treatment.

Clinical heterogeneities and prognoses of patients with myositis specific antibody negative dermatomyositis: a retrospective study in China.

OBJECTIVES: The clinical features of myositis specific antibody negative dermatomyositis (MSA negative DM) varied greatly, and there were few reports in the literatures. This study aimed to describe and expand the clinical phenotypes and prognoses of MSA negative DM patients. METHODS: MSA negative DM patients were identified from January 2010 to June 2020. We retrospectively reviewed the clinical features and laboratory data. The survival status was followed up until July 31. 2020 SPSS version 21.0 and R version 3.6.1 software were used for the statistical analyses. RESULTS: A total of 97 MSA negative DM patients were enrolled. The most common type of rashes was heliotrope rash (80.4%). More than half of the patients (55.7%) had interstitial lung disease (ILD), and seven of them developed rapid progressive ILD. There were eleven patients with tumours. During the follow-up, twelve patients died, of whom 5 (41.7%) died due to infection. Two phenotypes of MSA negative DM patients were identified by cluster analysis. Patients in cluster 1 developed muscle weakness, mechanic's hands, arthritis, and ILD more frequently. Patients in cluster 2 had a higher incidence of heliotrope rashes. Patients in cluster 1 tended to have worse prognoses, wherein the 1-year and 5-year survival rates (81.1% and 78.4%, respectively) were lower than those in cluster 2 (97.6% and 95.2%, respectively), with p value 0.04 and 0.056 respectively. CONCLUSIONS: Through cluster analysis, different clinical phenotypes of MSA negative DM patients were determined. The prognoses of the two subgroups were different in terms of survival rate and cause of death.

Clinical role of bronchoalveolar lavage in dermatomyositis-associated interstitial lung disease.

OBJECTIVE: To investigate the role of bronchoalveolar lavage (BAL) in DM-associated interstitial lung disease (ILD). METHODS: We retrospectively reviewed the medical records of patients with DM-ILD who underwent bronchoscopy between October 2015 and September 2019. We then collated clinical features, laboratory data and bronchoscopy findings. The follow-up study was terminated on the 1 May 2020. RESULTS: A total of 113 DM-ILD patients were included in this study, including 27 patients with acute/subacute interstitial pneumonia (A/SIP) and 86 patients with chronic interstitial pneumonia (CIP). The A/SIP group had significantly lower proportions of lymphocytes and eosinophils in the bronchoalveolar lavage fluid (BALF) than the CIP group, but had a significantly higher proportion of neutrophils. Pathogens were discovered in BALF from 28 (24.8%) patients. Twenty-five (22.1%) patients commenced or changed antibiotic therapy on the basis of their bronchoscopy results. Lymphopenia and intensive care unit care were significantly associated with pathogen-positive BALF findings. Complications of bronchoscopy occurred in nine (8.0%) patients; fever (5.3%) was the most common complication. Twenty-five deaths (25/106, 23.6%) were observed during a mean follow-up of 22 months. Age, A/SIP and anti-MDA5 antibody were identified as independent predictors of a poor outcome, while mechanic's hands was an independent protective factor. However, cellular and pathogen findings in BALF had no significant influence on 30-day or overall mortality. CONCLUSION: Bronchoscopy is a relatively useful instrument to evaluate ILD in patients with DM, and BAL can improve the diagnosis of infection. However, cellular and pathogen findings from BALF had no significant influence on prognosis.

The effects of infliximab in treating idiopathic inflammatory myopathies: A review article.

Anti-TNF treatment may be useful for patients with idiopathic inflammatory myopathies (IIMs). The purpose of this study is to assess the efficacy of infliximab (IFX) in the management of IIMs. Two databases (ie, PubMed and China National Knowledge Infrastructure) were searched up to Nov 2020 for studies investigating skin lesions and muscular weakness in patients with IIMs treated with IFX. A total of 18 studies were included. One hundred and eighteen patients were identified, including 58 adult patients and 60 patients with juvenile dermatomyositis (JDM) treated with IFX. Among these patients, 110 (93%) patients with refractory cases. In addition to glucocorticoids, patients from 15/18 studies received immunosuppressant agents (ISAs) concomitantly with IFX, among which methotrexate (MTX) was most common. After treatment with IFX, skin lesions and muscle strength were improved in 67% of patients with DM and 75% of patients with JDM, respectively. Skin calcinosis was improved in 21/34 (62%) of patients with JDM. Only 55% (12/22) of patients with polymyositis exhibited improvements in muscle strength. Lastly, 40% (42/104) of patients reported adverse events. Current evidence appears to support the use of IFX in some patients with refractory IIMs, especially those with JDM. The most common adverse reaction was infection. Large, randomized-controlled studies should be carried out to confirm these findings.

Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis responds to rituximab therapy.

OBJECTIVES: The purpose of this study was to assess the efficacy of rituximab (RTX) in the management of anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis (DM), with or without rapidly progressive interstitial lung disease (RP-ILD). METHODS: Medical records of DM patients with anti-MDA5 antibodies treated with RTX therapy were reviewed retrospectively. Skin rash data, lung function tests, chest high-resolution computed tomography (HRCT), and serum markers were compared before and after RTX. RESULTS: Eleven consecutive cases, including 5 males and 6 females, were identified. One hundred percent of patients had a typical DM rash and about 45% presented with skin ulceration. All the patients had ILD, 73% had RP-ILD, and 27% had mild or asymptomatic ILD. Ro-52 antibodies were found in 55% of this group. Lymphopenia was present in 10/11 patients (91%). Around half (55%) had a level of ferritin greater than 1000 ng/ml. Nine patients (82%) were refractory. These patients received intravenous RTX (375 mg/m2) at 0 and 14 days (conventional dose) or 100 mg once a week for 4 weeks (low dose). After RTX treatment, 2 patients (18%) with mild ILD showed complete remission, and 6 (55%) showed improvement in lung HRCT and/or lung function. Skin rash in 4 patients (100%) and ILD in 3 (75%) showed improvement in the low-dose group. Infection episodes occurred in four (57%) and one (25%) of the conventional-dose and low-dose group, respectively. CONCLUSIONS: Our study found that RTX is sufficient to improve skin rash and ILD or RP-ILD. Our results also suggest that lower RTX doses may be a useful therapy for anti-MDA5 antibody-positive DM patients. Key Points • To clarify efficacy of RTX in the management of anti-MDA5 antibody-positive DM, we performed a retrospective chart review of DM patients with anti-MDA5 antibodies who were treated with RTX. • This study found that RTX is sufficient to improve skin rash and ILD or RP-ILD. • The results suggest that low-dose RTX in treatment of MDA5-DM results in better responses and fewer adverse events.

Interstitial Lung Disease Is a Major Characteristic of Patients Who Test Positive for Anti-PM/Scl Antibody.

OBJECTIVE: This study aimed to analyze the clinical features of anti-PM/Scl antibodies in Chinese patients. METHOD: We reviewed the clinical data of anti-PM/Scl antibody-positive patients, including their long-term follow-up. RESULTS: A total of 30 patients carried anti-PM/Scl antibodies, 21 (70%) were females, and the mean age was 55.4 years, 15 (50%) and 10 (33.3%) patients were positive for anti-PM/Scl-75 and anti-PM/Scl-100, respectively. Fifteen cases (50%) were diagnosed as inflammatory myopathy, namely, 11 dermatomyositis (DM) and 4 polymyositis (PM). Five (16.7%) patients were diagnosed with overlap syndrome, and only one (3.3%) was diagnosed as systemic sclerosis. The other 9 (30%) patients were classified as undifferentiated connective tissue disease. Twenty-six (86.7%) had interstitial lung disease (ILD) and was the sole manifestation in 8 (26.7%) patients, 15 (58.0%) showed non-specific interstitial pneumonia based on high-resolution CT or lung biopsy. The majority of patients (95%) with mild and moderate groups on basis of pulmonary function tests. Compared to the anti-PM/Scl-100 group, the occurrence of clinical characteristics was not significantly different from the anti-PM/Scl-75 group, except the levels of C-reactive protein and erythrocyte sedimentation rate in the anti-PM/Scl-75 antibody-positive group were higher (p < 0.05). All patients with positive Ro-52 antibodies had ILD and were more likely to develop skin rash in the group with Ro-52 (p = 0.024). With a follow-up of the present cohort, 70.8% improved with treatment, but 16.7% of patients are easy to relapse. CONCLUSION: The anti-PM/Scl antibody occurred frequently in DM/PM patients, ILD was the major clinical feature, especially in patients combined with Ro-52. Some patients may complicate with ILD alone without extrapulmonary manifestations. Anti-PM/Scl antibodies positive patients were responsive to treatment.