External validation of the 2017 ACR/EULAR classification criteria for inflammatory myopathies in a Mexican cohort: Role of autoantibodies in the diagnosis and classification of patients with inflammatory myopathies.

OBJECTIVE: This retrospective study aimed to perform the first external validation of the ACR/EULAR classification criteria for inflammatory myopathy (IIM) in a Mexican dynamic cohort where the patients were evaluated with clinical and laboratory values. As secondary objectives, we presented the clinical characteristics of the patients and included antibodies other than anti Jo1 to evaluate their impact on our population. METHODOLOGY: This study included 70 patients with IIM and 70 patients with differential diagnoses of IIM, according to the absolute score of the classification criteria. We obtained sensitivity and specificity in the modality without biopsy, and as an exploratory analysis, we added other antibodies from the myositis extended panel. We analyzed the area under the curve (AUC) of three models: score without antibodies, with anti Jo1 and with any antibody. RESULTS: The ACR/EULAR criteria showed increased specificity and at least similar sensitivity to that of the original cohort (85% sensitivity and 92% specificity), with a cohort point of >55%. When we classified patients into definite, probable, possible, and no IIM categories, by adding the extended myopathy panel, 6 of the 10 patients initially classified as "no IIM" changed their classification to "Probable IIM" and 4 to "Definite IIM"; of the 16 patients classified as "probable IIM," 15 changed their classification to "Definite IIM." CONCLUSION: Considering the limitations of this study, we concluded that the 2017 EULAR/ACR criteria for IIM classification are sensitive and specific for classifying patients with IIM in the Mexican population. Additionally, the addition of antibodies other than anti-Jo1 may improve performance in certain populations.

External validation of the 2017 ACR/EULAR classification criteria for inflammatory myopathies in a Mexican cohort: Role of autoantibodies in the diagnosis and classification of patients with inflammatory myopathies / Validación externa de los criterios de clasificación de la ACR/EULAR de 2017 para miopatías inflamatorias en una cohorte mexicana. Papel de los anticuerpos en el diagnóstico y la clasificación de pacientes con miopatías inflamatorias

Objective: This retrospective study aimed to perform the first external validation of the ACR/EULAR classification criteria for inflammatory myopathy (IIM) in a Mexican dynamic cohort where the patients were evaluated with clinical and laboratory values. As secondary objectives, we presented the clinical characteristics of the patients and included antibodies other than anti Jo1 to evaluate their impact on our population. Methodology: This study included 70 patients with IIM and 70 patients with differential diagnoses of IIM, according to the absolute score of the classification criteria. We obtained sensitivity and specificity in the modality without biopsy, and as an exploratory analysis, we added other antibodies from the myositis extended panel. We analyzed the area under the curve (AUC) of three models: score without antibodies, with anti Jo1 and with any antibody. Results: The ACR/EULAR criteria showed increased specificity and at least similar sensitivity to that of the original cohort (85% sensitivity and 92% specificity), with a cohort point of >55%. When we classified patients into definite, probable, possible, and no IIM categories, by adding the extended myopathy panel, 6 of the 10 patients initially classified as “no IIM” changed their classification to “Probable IIM” and 4 to “Definite IIM”; of the 16 patients classified as “probable IIM,” 15 changed their classification to “Definite IIM.” Conclusion: Considering the limitations of this study, we concluded that the 2017 EULAR/ACR criteria for IIM classification are sensitive and specific for classifying patients with IIM in the Mexican population. Additionally, the addition of antibodies other than anti-Jo1 may improve performance in certain populations.(AU)

Objetivo: Este estudio retrospectivo tuvo como objetivo realizar la primera validación externa de los criterios de clasificación ACR/EULAR para miopatía inflamatoria (MII) en una cohorte dinámica de pacientes mexicanos que fueron evaluados en consulta y con muestras de laboratorio. Como objetivos secundarios presentamos las características clínicas de los pacientes e incluimos anticuerpos distintos al anti-Jo1 para evaluar su impacto en nuestra población. Metodología: Este estudio incluyó a 70 pacientes con MII y 70 pacientes con diagnóstico diferencial de MII, según la puntuación absoluta de los criterios de clasificación. Obtuvimos la sensibilidad y la especificidad en la modalidad sin biopsia, y como análisis exploratorio añadimos otros anticuerpos del panel extendido de miositis. Analizamos el área bajo la curva (AUC) de tres modelos: puntuación sin anticuerpos, con anti-Jo1 y con cualquier otro anticuerpo. Resultados: Los criterios ACR/EULAR mostraron una mayor especificidad y una sensibilidad, al menos similar a la de la cohorte original (85% de sensibilidad y 92% de especificidad), con un punto de cohorte de >55%. Cuando clasificamos a los pacientes en las categorías de definitiva, probable, posible y sin MII, al agregar el panel ampliado de miopatía, 6 de los 10 pacientes clasificados inicialmente como «Sin MII» cambiaron su clasificación a «Probable MII» y 4 a «MII Definitiva»; de los 16 pacientes clasificados como «Probable MII», 15 cambiaron su clasificación a «MII Definitiva». Conclusión: Considerando las limitaciones de este estudio, concluimos que los criterios de 2017 de la EULAR/ACR para la clasificación de la MII son sensibles y específicos para clasificar a los pacientes con MII en la población mexicana. Además, la adición de anticuerpos que no sean anti-Jo1 puede mejorar la estadificación en ciertas poblaciones.(AU)

Mycophenolate mofetil and mycophenolic acid for the treatment of eosinophilic fasciitis: report of two cases and literature review.

Eosinophilic fasciitis (EF) is an uncommon autoimmune connective tissue disorder characterized by edema, erythema, and subsequent induration of the extremities. It is commonly treated with corticosteroids but there is no treatment ladder for immunosuppressants or steroid-sparing agents. We report two EF cases treated effectively with mycophenolate mofetil (MMF) or mycophenolic acid (MPA) and present a literature review. We performed a MEDLINE search using the keywords 'eosinophilic fasciitis', 'Shulman syndrome', 'mycophenolic acid', or 'mofetil mycophenolate', and found 8 articles with 27 cases in which MMF or MPA was used. Twenty-nine cases were reviewed (2 reported herein and 27 from the literature search); all patients received a combination of systemic corticosteroids and MMF. MMF/MPA were given as a steroid-sparing agent in 27 (93.1%), in 1 (3.4%) as adjunctive therapy with other immunosuppressants, and in one, as monotherapy 1 (3.4%). Nineteen had a complete response, 6, a partial response, and 2 were unresponsive to diverse immunomodulators; in 2 cases, the outcome was not reported. MMF and MPA show promising therapeutic results and could be a treatment option to reduce corticosteroid related side effects.

Adverse events of six COVID-19 vaccines in patients with autoimmune rheumatic diseases: a cross-sectional study.

Data regarding COVID-19 vaccine efficacy and adverse events (AE) in patients with autoimmune and inflammatory rheumatic diseases (AIIRD) have been published recently although these mostly include the mRNA vaccines (Pfizer-BioNTech and Moderna) and the ChAdOx1 nCoV-19/AZD1222 (Oxford-AstraZeneca). This research aimed to study the prevalence of AE presented with six different SARS-CoV-2 vaccines {ChadOX1 nCoV-19 (AZD1222), Ad5-nCoV2, Ad26.COV2.S, mRNA-1273, BNT162b2, and CoronaVac} in Mexican patients with AIIRD. We performed a cross-sectional study about vaccine history. Two hundred and twenty five consecutive patients were recruited, mean age was 50.7 years and the majority (n = 213; 94.6%) were females. One hundred and seven (47.5%) received BNT162b2 mRNA, 34 (15.1%) Ad5-nCoV, 29 (12.8%) mRNA-1273, 28 (12.4%) ChAdOX1 nCoV-19 (AZD1222), 22 (9.7%) CoronaVac and 5 (2.2%) Ad26.COV2.S. The vaccines that had the most AE proportionally to the number of patients vaccinated were Janssen (5; 100%) followed by Pfizer-BioNTEch (86; 80%) and CanSinoBIO (27; 79.4%). Localized pain was the most frequent (158; 70.2%) AE. Fatigue (78; 34.7%), headache (69; 30.6%) and muscle ache (66; 29.3%) were the most common systemic symptoms. No serious AE that required medical attention or hospitalization were reported. The current results support the safety of different COVID-19 vaccines in patients with AIIRD. This information can help fight vaccine hesitancy in this population.