To characterize the clinical and serological correlates of cardiovascular magnetic resonance (CMR)-confirmed myocarditis in idiopathic inflammatory myopathies (IIM), evaluate the diagnostic performance of high-sensitivity cardiac troponin I (hs-TnI), and quantify the independent prognostic impact of myocarditis relative to interstitial lung disease (ILD). Single-center retrospective cohort study (STROBE-compliant) of 142 consecutive adults with IIM (2019-2025). Myocarditis was confirmed by CMR according to the 2018 Lake Louise Criteria, performed both at diagnosis and during follow-up (with or without immunosuppression) upon clinical suspicion and/or elevated hs-TnI. Myositis-specific antibodies (MSA), myositis-associated antibodies (MAA), anti-Ro52 and antiphospholipid antibodies (aPL, Sydney criteria: ≥40 GPL/MPL units confirmed at ≥ 12 weeks) were systematically recorded. Associations were assessed with Fisher's exact test and logistic regression; multivariable models were pre-specified as parsimonious (two predictors) given the limited event count. Overall survival was analyzed with Kaplan-Meier curves, the log-rank test and Cox proportional-hazards regression. Myocarditis was confirmed in 9/142 patients (6.3%), without significant association with IIM class (P = 0.303) or with the antisynthetase antibody subgroup (anti-Jo-1 + PL-12 + PL-7 + EJ; 5.8% vs. 6.7%, P = 1.000). In univariable analyses, myocarditis was associated with Raynaud phenomenon (OR 13.6, 95% CI 2.3-80.0), aPL positivity (OR 10.7, 95% CI 2.6-43.9), anti-Ro52 coexisting with MSA/MAA (OR 8.7, 95% CI 2.2-34.8), anti-PM/Scl (OR 7.1, 95% CI 1.6-30.3), fever (OR 7.1, 95% CI 1.6-30.3) and ILD (OR 4.7, 95% CI 1.1-20.3). hs-TnI was markedly higher in myocarditis (median 366 vs. 3.5 ng/L; P < 0.001) with an area under the receiver-operating characteristic curve (AUROC) of 0.91 (95% CI 0.83-0.98). In the event-constrained multivariable model, Raynaud phenomenon (adjusted OR 13.1, 95% CI 1.5-112.7) and aPL (adjusted OR 7.2, 95% CI 1.4-36.0) remained independently associated. All-cause mortality was higher in myocarditis (44% vs. 9%; OR 7.95, 95% CI 2.0-31.5; log-rank P = 0.030; univariable Cox HR 3.77, 95% CI 1.03-13.78; P = 0.044), and persisted after adjustment for ILD in the logistic model (adjusted OR 5.9, 95% CI 1.3-26.1; P = 0.020), whereas ILD attenuated to a non-significant trend (adjusted OR 2.9, 95% CI 0.9-9.2; P = 0.073). In this single-center retrospective cohort, IIM-associated myocarditis was consistently associated with Raynaud phenomenon, aPL positivity and anti-Ro52 coexistence, and was associated with higher mortality than ILD. These findings support hs-TnI-guided triage to CMR as part of systematic cardiac surveillance in IIM, pending multicenter confirmation.

Distinguishing autoimmune glomerulonephritis from hereditary nephropathy is critical to avoid unnecessary immunosuppression and ensure appropriate long-term renal monitoring. A 31-year-old Hispanic female patient with a complex medical history, including proteinase 3 (PR3)-positive granulomatosis with polyangiitis (GPA), rheumatoid arthritis, Raynaud phenomenon, seizure disorder, autism spectrum disorder, bipolar disorder with psychotic features, and cochlear implantation in childhood, was found to have genetically confirmed Alport syndrome through Natera testing (Natera, Inc., Austin, Texas, United States). Despite preserved renal function (estimated glomerular filtration rate (eGFR) 107 mL/minute/1.73m²), urinalysis demonstrated persistent proteinuria. Her history of early-onset hearing loss requiring cochlear implantation raised suspicion for hereditary nephropathy. Elevated anti-PR3 antibodies were present without clinical evidence of active vasculitis. The coexistence of autoimmune disease and hereditary collagen IV nephropathy complicated the attribution of urinary abnormalities. This case highlights the importance of genetic evaluation in patients with multisystem disease and urinary abnormalities, particularly when clinical features such as early-onset sensorineural hearing loss are present. Recognition of Alport syndrome in patients with coexisting autoimmune vasculitis has significant implications for renal surveillance, immunosuppressive stewardship, and multidisciplinary management.

Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) is a recognized extra-articular manifestation of rheumatoid arthritis. Pulmonary involvement may occasionally precede overt articular disease, creating diagnostic challenges. A patient presented with breathlessness for 2-3 months and joint pain of similar duration, along with bluish discoloration of the left middle finger, suggestive of Raynaud's phenomenon. Respiratory examination revealed bilateral inspiratory crepitations in the infrascapular and infra-axillary areas. Rheumatoid factor was positive, while antinuclear antibody was negative. High-resolution computed tomography (HRCT) of the thorax demonstrated microcystic honeycombing, basal subpleural fibrosis, and traction bronchiectasis, consistent with a usual interstitial pneumonia (UIP) pattern. This case highlights ILD as a potential early manifestation of rheumatoid arthritis and emphasizes the importance of autoimmune evaluation in patients presenting with fibrotic ILD and systemic features such as Raynaud's phenomenon.

We describe a 79-year-old man with stage IV non-small cell lung cancer previously treated with pembrolizumab who presented with progressive dyspnea and hypoxemia. Evaluation demonstrated bilateral ground glass opacities with interstitial changes compatible with a nonspecific interstitial pneumonia pattern, echocardiographic findings concerning for pulmonary hypertension, Raynaud phenomenon, and autoimmune serologies including elevated polymyositis/scleroderma 75 (PM/Scl 75) and small nuclear ribonucleoprotein 70 kilodalton (SNRNP 70Kd) antibodies suggestive of a scleroderma-like autoimmune process. Infectious evaluation, including blood and urine cultures and respiratory pathogen testing, did not identify a causative pathogen, although sputum culture, bronchoscopy with bronchoalveolar lavage, and lung biopsy were not performed, which is an important limitation. The patient was treated with broad-spectrum antibiotics, systemic corticosteroids, and ventilatory support, with planned initiation of additional immunosuppressive therapy. Despite these interventions, his respiratory status worsened, requiring mechanical ventilation, and his course was complicated by renal failure, heparin-induced thrombocytopenia, and multiorgan dysfunction. This case illustrates a rare, likely immune checkpoint inhibitor-associated scleroderma-like pulmonary syndrome in a patient with multiple comorbidities and underscores the need to consider progressive autoimmune disease among several potential etiologies in patients presenting with unexplained respiratory decline after immunotherapy, while acknowledging that a definitive causal relationship cannot be established in the absence of a complete diagnostic evaluation.

[This corrects the article DOI: 10.1093/jscr/rjaf535.].