RESUMEN

Background: Pulmonary infection is a common clinical complication associated with glucocorticoid. There have been no reported cases of mixed infections involving Nocardia and Pneumocystis jirovecii combined with anti-synthetase syndrome (ASS) activity. Methods: This study conducted a retrospective analysis of the clinical data from a patient with active ASS, treated for a pulmonary coinfection. Results: The patient exhibited fever, asthma, and cough as initial symptoms. Chest CT scan revealed multiple infiltration shadows, consolidation shadows, nodules, mass shadows, and internal cavities in both lungs. BALF mNGS detected Nocardia terpene and Pneumocystis jiroveci. Treatment with sulfamethoxazole/trimethoprim and corticosteroids led to an improvement. However, the patient experienced recurrent fever and a new rash with the reduction of the glucocorticoid dosage. Further investigation identified positive anti-Jo-1 and anti-Ro-52 antibodies and myogenic lesions on electromyography, which confirmed the diagnosis of ASS. Following treatment with immunoglobulin, methylprednisolone, and cyclosporine, the patient's condition significantly improved. Conclusion: Immunodeficiency patients are susceptible to opportunistic infections. mNGS is valuable for diagnosis and treatment. Although the image of Nocardia terpene and Pneumocystis jiroveci infections lack specificity, they exhibit distinctive features. Should fever and skin lesions reoccur post-effective anti-infective therapy, it is imperative to explore non-infectious causes and expedite autoantibody testing.

RESUMEN

BACKGROUND: Anti-Ro-52 antibodies have been associated with interstitial lung disease (ILD) in various autoimmune diseases. However, their role in ILD among patients with idiopathic inflammatory myopathies (IIMs) is relatively underexplored. This study aimed to investigate the association between anti-Ro-52 antibodies and the occurrence of ILD in individuals with IIMs. METHODS: This retrospective observational study included 604 patients who underwent myositis autoantibody testing between July 2018 and January 2021 at our hospital and were diagnosed with either IIMs or IIM-mimics. Comparative analyses were conducted between IIMs and IIM-mimics, as well as within the IIM group between cases with and without ILD. Logistic regression or Firth's logistic regression analyses were employed to assess the risk of ILD development in different IIM subgroups and myositis antibody categories. RESULTS: This study included 190 patients with IIM and 414 patients with IIM-mimics. Patients with IIM demonstrated higher incidence of ILD, concurrent autoimmune disease, and a greater likelihood of various myositis autoantibodies when compared to the IIM-mimics group. Within the IIM patient cohort, those with ILD exhibited a later age of onset of IIM, an increased mortality rate, and a more frequent presence of anti-aminoacyl-tRNA synthetase (ARS) antibodies compared to those without ILD. The presence of any myositis-specific antibody (MSA) was associated with a six-fold increased risk of ILD, while dual positivity for MSA and anti-Ro-52 antibodies conferred a twenty-fold risk. Anti-ARS antibodies carried a 14-fold increased risk of ILD, which escalated to 38-fold in cases of dual positivity for anti-ARS and anti-Ro-52 antibodies. Anti-Ro-52 antibodies alone increased the risk eight-fold. CONCLUSIONS: Among patients with IIM, the presence of ILD was linked to higher mortality. Certain autoantibodies, notably anti-ARS and anti-Ro-52 antibodies, were associated with an increased risk of ILD. The greatest risk of ILD was observed in cases of dual positivity for anti-ARS and anti-Ro-52 antibodies.

Asunto(s)

RESUMEN

Interstitial lung disease is a common complication of anti-synthetase syndrome (ASS), and lymphocytic infiltration is often observed in the lesion. We have recently reported that disease-specific autoantibodies are produced by infiltrating lymphocytes in some autoimmune diseases. Here, we investigate the antigen specificity of B cells in the lung lesions of ASS patients. A total of 177 antibodies were produced from antibody-secreting cells in bronchoalveolar fluid (BALF) of three each of serum anti-Jo-1 and serum anti-EJ antibody-positive patients. Twelve to 30% and 50 to 62% of these antibodies were disease-specific autoantibodies, respectively. These autoantibodies recognized conformational epitopes of the whole self-antigen and had affinity maturations, indicating that self-antigens themselves are the target of humoral immunity. In addition, 100 antibodies were produced from two salivary gland tissues, obtained by chance, of ASS patients. Salivary glands are not generally recognized as lesions of ASS, but unexpectedly, ASS-related autoantibody production was also observed similar to that of BALF. Immunostaining confirmed the presence of ASS-related autoantibody-producing cells in salivary glands. Our results suggest that disease-specific autoantibody production at lesion sites is a common pathogenesis of autoimmune diseases, and that tissue-specific production of autoantibodies can provide insights regarding the distribution of organ manifestations in autoimmune diseases.

Asunto(s)

RESUMEN

This case report highlights dermatomyositis (DM) characterised by the concurrent presence of anti-melanoma differentiation-associated protein 5 (anti-MDA5) and anti-Ro52 antibodies. A 64-year-old woman initially presented with erythema on the palms, which later spread to the dorsum of the hands, followed by involvement of the face, forehead, and upper eyelids. The patient reported joint pain, fatigue, and dyspnea. Physical examination revealed characteristic cutaneous manifestations, including heliotrope rash and Gottron's sign, accompanied by skin ulceration and muscle weakness. Blood tests showed elevated levels of creatine phosphokinase and C-reactive protein. A high-resolution computed tomography (HRCT) scan revealed interstitial lung disease (ILD) with an organising pneumonia (OP) pattern. Magnetic resonance imaging (MRI) confirmed the presence of myositis. Autoantibody analysis revealed concurrent positivity for both anti-MDA5 and anti-Ro52 antibodies. At the time of diagnosis, she had no respiratory impairment, but had an elevated C-reactive protein and high levels of anti-MDA5 antibody. She was started on triple combination therapy with glucocorticoids, cyclophosphamide, and tacrolimus. She had worsening oxygenation and elevated ferritin during the first weeks of treatment, but then her symptoms improved. Early detection of a co-positive anti-Ro52 antibody led to early initiation of triple combination therapy and a good prognosis.

Asunto(s)

RESUMEN

OBJECTIVES: To clarify clinical features of anti-Ro52 antibody (Ab)-positive polymyositis (PM)/dermatomyositis (DM). PATIENTS AND METHODS: We retrospectively examined clinical features and status of anti-Ro52 Ab in patients with PM/DM admitted at the University of Tsukuba Hospital between January 2019 and February 2023. We compared anti-Ro52 Ab-positive and -negative groups. RESULTS: A total of 40 patients were selected and analyzed. Median age at diagnosis was 61.5 (48.8-69.3) years and 34 cases were female. Twenty-three cases were PM and 17 cases were DM (including 6 clinically amyopathic dermatomyositis: CADM). Twenty-two cases were positive for anti-Ro52 Ab, 14 for anti-ARS Ab, and 6 for anti-MDA5 Ab. Interstitial lung disease (ILD) was detected in 29 cases, 9 of which were rapidly progressive. Glucocorticoid (GC)-resistant cardiomyopathy was detected in 6 cases, malignancy in 3 cases, and Sjögren's syndrome (SS) in 4 cases. Of the 22 anti-Ro52 Ab positive cases, only 3 were single-positive and the remaining 19 cases simultaneously had other autoantibodies. Comparing the anti-Ro52 Ab-positive and -negative groups, the frequencies of anti-ARS Ab positivity (63.6% vs. 0%), ILD (95.5% vs. 44.4%), GC-resistant cardiomyopathy (27.3% vs. 0%), concomitant use of immunosuppressants (95.5% vs. 55.6%), and levels of C-reactive protein (CRP) were significantly higher in the anti-Ro52 Ab-positive group (p<0.05). The frequencies of PM/DM, positivity of anti-MDA5 Ab, malignancies, and SS were comparable between groups. CONCLUSION: Anti-Ro52 Ab were frequently positive in PM/DM and anti-Ro52 Ab-positive patients showed significantly higher rates of anti-ARS Ab positivity and ILD, GC-resistant cardiomyopathy, concomitant use of immunosuppressants, and higher levels of CRP. Anti-Ro52 Ab may be useful as a severity marker in PM/DM.

RESUMEN

Objective:To explore clinical factors of poor prognosis in patients with anti-melanoma differentiation-associated gene 5 andtibody positive dermatomyositis (MDA5-DM).Methods:One hundred and twenty-six enrolled adults with MDA5-DM were divided into the survival group and the deceased group according to the outcomes. Survival time, clinical manifestations, laboratory tests, pulmonary function tests, myositis antibodies and treatments were collected for statistical analysis. Serum concentrations of IL-15, HMGB1, and sCD163 were measured by ELISA in MDA5-DM patients and healthy controls. Mann-Whitney U nonparametric test and Student′s t-test were used to compare the continuous variables between the two groups, and χ2 or Fisher′s exact test were used for comparison of categorical variables. Cox regression analysis was used to assess the survival predictors in MDA5-DM patients. The cumulative survival rate was calculated by Kaplan-Meier curve analysis, and Log-rank tests were used to examine differences in survival curves. P<0.05 was considered statistically significant. Results:Cox multivariate regression analysis revealed that age > 57 years [ HR (95% CI)=3.05 (1.20, 7.80), P=0.020], RP-ILD [ HR (95% CI)=25.07 (5.42, 115.98), P<0.001], and levels of anti-Ro52 antibody [ HR (95% CI)=3.41 (1.36, 8.53), P=0.009] were important prognostic factors independent of multiple clinical parameters. The ELISA test results showed that the levels of serum IL-15[0.91 (0.66, 2.00)pg/ml vs. 0.51(0.39, 0.72)pg/ml, Z=-4.57, P<0.001] and HMGB1 [230.53(90.40, 394.31)ng/ml vs. 32.66 (17.82, 46.21)ng/ml, Z=-6.52, P<0.001] in MDA5-DM patients were significantly higher than those in healthy controls, but there were no significant differences in the level of serum IL-15 [1.21(0.63, 2.12)pg/ml vs. 0.91(0.68, 1.66)pg/ml, Z=-0.30, P=0.766], HMGB1[267.61(167.03, 444.23)ng/ml vs. 228.35(74.74, 344.32)ng/ml, Z=0.82, P=0.413], and sCD163 [112.70(93.45, 148.51)ng/ml vs. 132.72(96.79, 203.18)ng/ml, Z=-0.62, P=0.536] between the survival group and the deceased group. Conclusion:Older age, RP-ILD, and high levels of anti-Ro52 antibody significantly increase the risk of death in MDA5-DM patients. Intensive follow-up of patients with the above factors in the early stages may help to improve the prognosis.

RESUMEN

Protein tripartite motif-containing 21 (TRIM21/Ro52), an E3 ubiquitin ligase, is an essential regulator of innate immunity, and its dysregulation is closely associated with the development of autoimmune diseases, predominantly systemic lupus erythematosus (SLE) and primary Sjögren's syndrome (pSS). TRIM21 /Ro52 also features anti-cancer and carcinogenic functions according to different malignancies. The interconnected role of TRIM21/Ro52 in regulating autoimmunity and cell metabolism in autoimmune diseases and malignancies is implicated. In this review, we summarize current findings on how TRIM21/Ro52 affects inflammation and tumorigenesis, and investigate the relationship between TRIM21/Ro52 expression and the formation of lymphoma and breast cancer in SLE and pSS populations.

RESUMEN

BACKGROUND: This study aimed to describe the clinical characteristics and analyze the poor prognostic factors in patients with anti-MDA5 dermatomyositis. METHODS: A total of 126 adults with anti-MDA5 dermatomyositis were enrolled in this retrospective study. Information on survival time, cause of death, and baseline characteristics was collected. Patients were divided into two groups: a survival group and a non-survival group. Items with clinical significance that showed significant differences between the two groups were screened by Kaplan-Meier and Cox regression analyses to identify the predictors of poor survival. RESULTS: Thirty-two patients were included in the non-survival group, most of whom died from respiratory failure, with pulmonary infection accounting for half. Epstein-Barr virus infection was relatively common in both groups. Aspartate transaminase, lactate dehydrogenase, and ferritin levels; erythrocyte sedimentation rate; and anti-Ro52 antibody levels were significantly higher, while the lymphocyte count was lower in the non-survival group compared with the survival group. Notably, patients in the non-survival group were more likely to present with rapidly progressive interstitial lung disease than those in the survival group. Kaplan-Meier and Cox multivariate regression analyses revealed that the prevalence of rapidly progressive interstitial lung disease, levels of anti-Ro52 antibody, and age > 57 years were important prognostic factors independent of multiple clinical parameters. CONCLUSIONS: Rapidly progressive interstitial lung disease, anti-Ro52 antibody levels, and age > 57 years are possible predictors of mortality risk in patients with anti-MDA5 dermatomyositis.

Asunto(s)

RESUMEN

A 65-year-old female was admitted with rapidly progressive respiratory failure requiring intubation and mechanical ventilation. She was considered to have an infective exacerbation of underlying interstitial lung disease (ILD). She improved on antibiotics, but the interstitial process progressed rapidly, and she could not be weaned. An antimyositis antibody panel yielded a strongly positive anti-Jo-1 and anti-Ro 52. A diagnosis of antisynthetase syndrome (ASS) associated ILD, a very rare disease with high mortality, was made. She was managed with high-dose corticosteroids and intravenous immunoglobulin therapy and was eventually liberated from mechanical ventilation. This case highlights the importance of considering ASS in an otherwise unexplained rapidly progressive ILD requiring mechanical ventilation.

RESUMEN

OBJECTIVES: Interstitial lung disease (ILD) is common in anti-synthetase syndrome (ASS). Progressive fibrosing ILD (PF-ILD) may develop in ILD with autoimmune features. Data on PF-ILDs in ASS as a group are scarce. This study aimed to explore the characteristics and predictors of PF-ILD in ASS patients. METHODS: This retrospective study enrolled 96 ASS-ILD patients. Baseline clinical data were collected. PF-ILD assessments were conducted at every hospital visit during windows of 24 months after initial diagnosis. Phenotypic, survival features and predictors of PF-ILD were estimated through SPSS 22.0. RESULTS: The results revealed that 35.42% (34/96) were evaluated to be PF-ILD with a median interval time of 14.73 months. Nonspecific interstitial pneumonia was the most common radiological pattern of PF-ILD. Ground glass opacity (GGO), traction bronchiectasis and reticulation were representative high-resolution computed tomography findings of this group. Compared with the non-progressive group, PF-ILD patients had higher frequencies of anti-Ro-52 antibodies (91.18% vs 66.13%, P = 0.007) and GGO in the lower + middle and lower + middle + upper zones of the left lung, as well as lower + middle zones in the right lung (85.30% vs 54.84%, P = 0.003; 64.71% vs 38.71%, P = 0.015; 82.35% vs 58.06%, P = 0.016). Multivariate Cox analysis identified that anti-Ro-52 antibody (hazards ratio [HR] 3.55, 95% CI 1.06-11.90, P = 0.040) and GGO in left lower + middle lung zones (HR 22.11, 95% CI 1.95-250.90, P = 0.012) were independent risk factors for PF-ILD. CONCLUSIONS: PF-ILD was associated with poor prognosis. Over one-third of ASS-ILD patients may develop to PF-ILD. Anti-Ro-52 antibody positivity and GGO in left lower + middle lung zones were independent risk factors for PF-ILD in ASS patients.

Asunto(s)

RESUMEN

Anti-MDA5 antibody dermatomyositis (DM) is a special type of myositis, which can potentially cause rapidly progressive interstitial lung disease (RP-ILD). Mixed connective tissue disease (MCTD) is a complex disease with different characteristics of autoimmune connective tissue disease, associated with ILD. Both are rare diseases, and few patients with both diseases have been reported. A 71-year-old woman complained of palpitations, with a 2 months history of rash around her hands, extensor surface of right elbow, and the nape of her neck. Subsequently, the patient had acute exacerbation of dyspnea and tachypnea. Anti-Ro52, U1 RNP and MDA5 antibodies were positive; the presenting evidence was suggestive of anti-MDA5+ DM-RP-ILD complicated with MCTD. Our patient deteriorated rapidly and had a fatal outcome, despite "triple therapy" for RP-ILD. This case illustrates that patients with coexisting anti-MDA5+ DM and MCTD have the former's typical clinical manifestations, and may develop ILD quickly rather than slowly as in MCTD, especially with the coexistence of anti-Ro52 antibodies.

Asunto(s)

RESUMEN

OBJECTIVE: To summarize the clinical, serological, and radiological characteristics of anti-synthetase syndrome (ASS) patients with different anti-aminoacyl-tRNA synthase antibody. METHODS: Retrospective analysis was performed based on the clinical data of 88 patients diagnosed with ASS in Tianjin Medical University General Hospital from January 2015 to December 2020. The clinical data included general conditions, serological indexes, high-resolution CT (HRCT) characteristics, and pulmonary function manifestations. RESULTS: Among the 88 patients, there were 17 males and 71 females. The anti-synthetase antibodies included anti-Jo-1 (n = 42), anti-PL-7 (n = 14), anti-PL-12 (n = 9), anti-EJ (n = 20), and anti-OJ (n = 3) antibodies. The most common clinical manifestations of ASS patients were interstitial lung disease (ILD) (90%, 79/88), followed by myositis (79.5%, 70/88), arthritis (50%, 44/88), and rash (50%, 44/88). The frequency of arthritis in the anti-Jo-1 antibody-positive group was higher than that of the anti-PL-7 and anti-EJ antibody groups (P = 0.004, P = 0.002, respectively). The frequency of Gottron's sign in the anti-PL-7 antibody positive group was higher than that of the anti-Jo-1 and anti-PL-12 antibody-positive groups (P = 0.006, P = 0.04). Isolated arthritis was the most frequent initial symptoms in anti-Jo-1 antibody-positive group (47.6%, 20/42), while isolated ILD was most frequent in patients with anti-EJ antibody (50%, 10/20), and isolated myositis in patients carrying anti-OJ (66.7%, 2/3). There were only 32 cases (36.4%) with the typical clinical triad (myositis, arthritis, ILD). In our cohort, 79 patients (90%) were complicated with ILD. Meanwhile, 7 out of 79 cases were classified into rapid progressive group with 6 cases (85.7%) carrying anti-Ro-52 antibody. The probability of reticular and honeycombing shadow in HRCT of patients with anti-EJ antibody positive was higher than that of other groups (P < 0.05). CONCLUSION: ILD, myositis, and arthritis were the most common clinical manifestations in patients with ASS. Different antibody-positive patients have different initial symptoms. Patients with isolated arthritis, myositis, and ILD should be vigilant of ASS. The complication of anti-Ro-52 antibody in ASS patients was associated with rapidly progressive pulmonary interstitial disease. Patients with positive anti-EJ antibodies tend to have ILD as the first symptom, and with high occurrence of ILD, the HRCT showed more serious patterns, suggesting the correlation between anti-EJ antibodies and ILD. Key Points • Analyzing specific clinical manifestations in ASS patients with different ARS antibodies can raise awareness of the disease and reduce misdiagnosis. • Anti-EJ antibodies were correlated with ILD. • Anti-Ro-52 antibodies may correlate with RP-ILD in ASS patients.

Asunto(s)

RESUMEN

Objective To investigate the clinical features and prognosis of melanoma differentiation associated protein-5(MDA5)antibody and anti-Ro-52 antibody in double-positive dermatomyositis.Methods Forty-seven dermatomyositis patients with anti-MDA5 antibody positive admitted to the Second Affiliated Hospital of Air Force Military Medical University,Tangdu Hospital from August 2018 to July 2022 were collected.According to whether anti-Ro-52 antibody was positive,they were divided into MDA5 + Ro-52 pos-itive group(n =23)and MDA5 + Ro-52 negative group(n =24).The clinical data of the two groups were retrospectively analyzed,and the differences in the clinical characteristics,laboratory indicators,incidence of rapidly progressive interstitial lung disease and mortality between the two groups were compared.Results Compared between the two groups,the incidence of Gotton rash and hoarseness in the MDA5 + Ro-52 positive group was higher than that in the MDA5 + Ro-52 negative group,and the difference was statistically significant(P<0.05).There were no significant difference in the incidence of skin ulcers,periapillary erythema,positive rash,cape sign,fever,joint pain and sore throat(P>0.05).Lymphocyte count[0.65(0.50,0.81)×109/L vs 1.18(0.91,1.63)×109/L,z =-3.821,P =0.001]and serum albumin[33.40(29.40,35.67)g/L vs 37.25(32.65,40.27)g/L,z =-3.325,P =0.001],oxygen partial pressure[66.60(58.60,86.80)mmHg vs 88.60(75.67,95.72)mmHg,z =-2.373,P = 0.018],blood oxygen saturation[90.40%(89.00%,95.00%)vs 94.90%(90.50%,97.73%),z =-2.353,P = 0.019]in MDA5 + Ro-52 positive group were lower than those in MDA5 + Ro-52 negative group,and the difference were statistically significant(P<0.05).Erythrocyte sedimenta-tion rate[41.00(30.00,62.50)mm/h vs 28.50(21.50,48.75)mm/h,z =2.161,P =0.031]and serum lactate dehydrogenase lev-els[426.00(335.50,605.50)U/L vs 260.00(217.50,373.25)U/L,z =3.313,P =0.011],serum ferritin level[1210.00(465.50,2749.00)μg/L vs 366.00(150.25,629.25)μg/L,z =2.856,P =0.004],the incidence of rapidly progressive interstitial lung disease(73.91%vs 25.00%,χ2 =11.245,P =0.001)and mortality(43.47%vs8.33%,χ2 =7.630,P =0.006)in MDA5 + Ro-52posi-tive group were higher than those in anti-MDA5 + Ro-52 negative group,and the differences were statistically significant(P<0.05).Conclusion Dermatomyositis patients with double-positive anti-MDA5 antibody and anti-Ro-52 antibody are more likely to have increased serum serum lactate dehydrogenase and serum ferritin,decreased serum albumin and peripheral blood lymphocyte count,and more likely to be complicated with rapidly progressive interstitial lung disease and hypoxemia.The prognosis is poor and the mortality is high,which should be paid attention to by clinicians.

RESUMEN

Background: Anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis with interstitial lung disease (anti-MDA5 DM-ILD) is a disease with high mortality. We sought to develop an effective and convenient prediction tool to estimate mortality risk in patients with anti-MDA5 DM-ILD and inform clinical decision-making early. Methods: This prognostic study included Asian patients with anti-MDA5 DM-ILD hospitalized at the Nanjing Drum Hospital from December 2016 to December 2020. Candidate laboratory indicators were retrospectively collected. Patients hospitalized from 2016 to 2018 were used as the discovery cohort and applied to identify the optimal predictive features using a least absolute shrinkage and selection operator (LASSO) logistic regression model. A risk score was determined based on these features and used to construct the mortality risk prediction model in combination with clinical characteristics. Results were verified in a temporal validation comprising patients treated between 2019 and 2020. The primary outcome was mortality risk within one year. The secondary outcome was overall survival. The prediction model's performance was assessed in terms of discrimination, calibration, and clinical usefulness. Results: This study included 127 patients, (72 men [56.7%]; median age, 54 years [interquartile range, 48-63 years], split into discovery (n = 87, 70%) and temporal validation (n=37, 30%) cohorts. Five optimal features were selected by LASSO logistic regression in the discovery cohort (n = 87) and used to construct a risk score, including lymphocyte counts, CD3+CD4+ T-cell counts, cytokeratin 19 fragment (CYFRA21-1), oxygenation index, and anti-Ro52 antibody. The retained predictive variables in the final prediction model were age, Heliotrope, fever, and risk score, and the most predictive factor was the risk score. The prediction model showed good discrimination (AUC: 0.915, 95% CI: 0.846-0.957), good calibration (Hosmer-Lemeshow test, P = 0.506; Brier score, 0.12), and fair clinical usefulness in the discovery cohort. The results were verified among patients in the temporal validation cohort (n = 38). We successfully divided patients into three risk groups with very different mortality rates according to the predictive score in both the discovery and validation cohorts (Cochran-Armitage test for trend, P < 0.001). Conclusions: We developed and validated a mortality risk prediction tool with good discrimination and calibration for Asian patients with anti-MDA5 DM-ILD. This tool can offer individualized mortality risk estimation and inform clinical decision-making.

Asunto(s)

RESUMEN

Background: Programmed cell death protein-1/programmed cell death ligand-1 (PD-1/PD-L1) inhibitors therapy is now a routine scheme in cancers. However, the effect of preexisting autoantibodies on the safety and efficacy of PD-1/PD-L1 inhibitors in cancer patients is not well understood. Methods: The present retrospective cohort study evaluated the safety and efficacy of PD-1/PD-L1 inhibitors in patients with preexisting autoantibodies. Patients who received PD-1/PD-L1 inhibitors in the Department of Medical Oncology, Peking Union Medical College Hospital between November 2017 and August 2021 were reviewed. Results: 67 (37.9%) of the 177 patients, 27 (20.3%) of the 133 patients, and 16 (11.0%) of 146 patients who received PD-1/PD-L1 inhibitors were positive for ANA, anti-Ro52, and antithyroid antibodies, respectively. Preexisting ANA and anti-Ro52 antibody were not associated with the increased risk of immune-related adverse events (irAEs), while thyroid dysfunction was more frequent in patients with positive antithyroid antibody (75.0% versus 13.8%, p < 0.001). The median progression-free survival (PFS, 13.1 versus 7.0 months, p = 0.015) was significantly longer in the ANA-positive patients, while the median overall survival (OS, 14.5 versus 21.8 months, p = 0.67) did not differ significantly between the ANA-positive and ANA-negative groups. Moreover, the preexisting anti-Ro52 and antithyroid antibodies were not significantly associated with PFS and OS. Conclusions: The presence of ANA and anti-Ro52 antibody were not associated with a higher risk of irAEs, whereas patients positive for antithyroid antibody should monitor closely immune-related thyroid dysfunction. Preexisting ANA might be a predictor of longer PFS, while anti-Ro52 and antithyroid antibodies had no significant effect on survival outcomes in patients receiving PD-1/PD-L1 inhibitors therapy.

Asunto(s)

RESUMEN

BACKGROUND: Pulmonary fibrosis is common in primary Sjögren's syndrome (pSS)-related interstitial lung disease (ILD). However, research is lacking on the diagnostic immunological examination of pSS-related pulmonary fibrosis. Particularly, the value of detecting anti-Ro52 antibody in pulmonary fibrosis is unclear. OBJECTIVE: To evaluate the potential diagnostic value of anti-SSA, anti-SSB, and anti-Ro52 autoantibodies as markers of pSS-related pulmonary fibrosis. METHODS: One-hundred seventy-nine patients with pSS were analyzed retrospectively at our hospital. They were divided into the fibrosis and non-fibrosis groups. Pulmonary fibrosis was classified as mild, moderate, or severe based on the patients' computed tomography (CT) findings. Laboratory examinations, including anti-SSA, anti-SSB, and anti-Ro52 antibody evaluations, were performed. The influencing factors of pulmonary fibrosis were analyzed using logistic regression. RESULTS: Chest CT revealed pulmonary fibrosis in 45 patients with pSS (25.1%). The positive rates of anti-SSA and anti-Ro52 antibodies in the fibrosis group were lower than in the non-fibrosis group (P=0.04, P=0.001). The frequency of anti-Ro52 antibody showed no significant differences between mild-to-moderate (53.8%) and severe (47.3%) pulmonary fibrosis. The anti-Ro52 antibody was identified as a potentially protective factor against pSS (P=0.041). CONCLUSIONS: Patients with pSS and pulmonary fibrosis had a low frequency of anti-SSA and anti-Ro52 antibodies. In patients with pSS and negative anti-Ro52 antibody, a chest CT is recommended to further understand the patients' condition.

Asunto(s)

RESUMEN

Background: This study aimed to diagnose and treat a patient with anti-Ro52-positive antisynthetase syndrome (ASS), investigate the association between anti-Ro52 antibodies and ASS, and determine its clinical significance. The objective of this clinical report is to highlight this unusual syndrome to avoid incorrect diagnosis. Case Description: A middle-aged woman presenting with obvious lung symptoms was admitted to our hospital. A physical examination revealed swollen joints in both hands, mechanic's hands, and normal muscle strength and muscle tone in all 4 extremities. A myositis-specific antibody panel, lung computed tomographic (CT) imaging, electromyography, and muscle biopsy were performed as auxiliary examinations, and appropriate treatment was administered after the confirmed diagnosis. The myositis-specific antibody panel yielded strongly positive results for anti-Jo-1 and anti-Ro52 antibodies, lung CT imaging revealed interstitial lung disease, electromyography revealed myogenic damage, and muscle magnetic resonance imaging revealed multiple inflammatory exudates. A definitive diagnosis of ASS was made, and glucocorticoid and immunosuppressant therapy were administered. After treatment, the patient's symptoms were alleviated, creatine kinase activity was reduced, and signs of disease activity and secondary tumors were not observed on a subsequent follow-up evaluation. Conclusions: Anti-Ro52 antibodies, being myositis-associated antibodies, can lead to an atypical clinical presentation in ASS patients and are potentially associated with a poor prognosis. Therefore, thorough follow-up evaluation is required for such cases.

RESUMEN

OBJECTIVES: In the present study, we aimed to assess the prevalence and clinical significance of anti-Ro52 antibodies in a cohort of patients with idiopathic inflammatory myopathy-associated interstitial lung disease (IIM-ILD) with different myositis-specific autoantibodies (MSAs). METHODS: A cohort of 267 IIM-ILD patients, including 62 patients with PM, 126 patients with DM and 79 patients with clinically amyopathic DM (CADM) were retrospectively analysed in this study. Clinical and laboratory findings, pulmonary function tests (PFTs), HRCT patterns and treatment information were compared between patients with and without anti-Ro52 antibodies. The association between prognosis and anti-Ro52 antibodies was also evaluated based on different MSA subgroups. RESULTS: Anti-Ro52 antibodies were more frequent in patients with anti-MDA5 (62.1%, P < 0.01) and anti-Jo1 (64.9%, P < 0.01) antibodies than in those with other MSAs. The proportion of patients with anti-Jo1 antibodies was higher in the anti-Ro52 antibody-positive group than in the anti-Ro52 antibody-negative group. Patients with anti-Ro52 antibodies were more likely to exhibit the Gottron sign than the anti-Ro52 antibody-negative group (P < 0.001). Furthermore, it was a predictive factor for rapid progression interstitial lung disease (RP-ILD) (P = 0.001) and was also associated with a higher mortality rate (log-rank test, P = 0.001). Furthermore, RP-ILD was more frequently exhibited in anti-MDA5- and anti-Ro52-positive patients. Moreover, anti-Ro52 antibody positivity was closely associated with a higher mortality rate in anti-MDA5-ILD patients (log-rank test, P < 0.05). CONCLUSIONS: Anti-Ro52 antibodies were highly prevalent in patients with anti-MDA5 and anti-Jo1 antibodies. Within all patients with IIM-ILD, those with anti-Ro52 autoantibodies had a higher frequency of RP-ILD and a poorer prognosis, especially in the anti-MDA5 antibody subgroup.

Asunto(s)

RESUMEN

To analyze the clinical characteristics of patients with antisynthetase syndrome (ASS) and positive anti-Ro52 antibody. The clinical data of 203 ASS patients admitted to the First Affiliated Hospital of Zhengzhou University from 2017 to 2020 were analyzed retrospectively. Demographics, clinical manifestations, laboratory results, treatment and outcome were collected including data of 18 patients with rapidly progressive interstitial lung disease (RP-ILD). In total, the majority were women (148,72.9%). The average onset age was (51.9±13.3) years. There were 163 (80.3%) patients with positive anti-Ro52 antibody. The positivity in women (77.3% vs. 55.0%, P=0.004) was higher, and the median time from disease onset to diagnosis [4.5 (2.0, 24.0) months vs. 2.0 (1.0, 12.0) months, P=0.024] was longer in patients with positive anti-Ro52 antibody than those negative. Compared with negative patients, patients with positive anti-Ro52 antibody had a higher incidence of interstitial lung disease (ILD) (96.9% vs. 65.0%, P<0.001), arthritis (33.7% vs. 17.5%, P=0.046), and arthralgia (39.3% vs. 20.0%, P=0.022). Higher rate of positve antinuclear antibody (ANA) (85.3% vs. 55.0%, P<0.001), lower rate of positive anti-Jo-1 antibody (32.5% vs. 50.0%, P=0.039), lower albumin level [(34.6±5.2) g/L vs. (37.3±4.7) g/L, P=0.004] and lower lymphocyte counts [(1.4±0.8) ×10 9/L vs. (1.8±0.8) ×10 9/L, P=0.014] were more common in patients with positive anti-Ro52 antibody. The presence of anti-Ro52 antibody is associated with a particular phenotype of ASS, leading to common ILD, involvement of joints, high ANA positivity, low albumin and low lymphocyte counts.