Systemic sclerosis-associated interstitial lung disease (SSc-ILD) is a significant complication that impairs lung function and increases morbidity. Sarcopenia, characterized by the loss of muscle mass and function, is commonly found in chronic diseases such as systemic sclerosis. This study aimed to evaluate the prevalence of sarcopenia in patients with SSc-ILD and investigate its cross-sectional associations with pulmonary function test parameters, estimated pulmonary artery pressure and quantitative radiological lung involvement. In this cross-sectional study conducted between 2024 and 2025, 40 patients with systemic sclerosis (2013 ACR/EULAR criteria) and high-resolution computed tomography-confirmed interstitial lung disease were evaluated. Patients with overlap myositis, acute infections, severe orthopedic/neurological limitations, and end-stage organ failure were excluded. Sarcopenia was diagnosed based on European Working Group on Sarcopenia in Older People 2 criteria using muscle mass (bioelectrical impedance analysis) and handgrip strength measurements. Pulmonary function tests (FVC, single-breath transfer factor of lung for carbon monoxide), echocardiographic sPAP estimations, and quantitative high-resolution computed tomography lung density analysis were performed. These findings highlight the potential impact of sarcopenia on lung function and the association of sarcopenia with radiological findings in SSc-ILD SSc-ILD. Among the 40 analyzed patients (mean age: 55.8 ± 10.8 years, 85.0% female, mean BMI: 28.7 ± 5.0 kg/m2), sarcopenia was identified in 10 patients (25.0%). Patients with and without sarcopenia were compared to analyze its effects on pulmonary function and radiological findings. Patients with sarcopenia had significantly lower lung diffusion capacity (transfer factor of lung for carbon monoxide) and forced vital capacity % values and significantly higher radiological lung involvement compared to those without sarcopenia (P = .004, P = .023, and P = .035, respectively). Systolic pulmonary artery pressure was also significantly higher in sarcopenic patients (P = .031). Additionally, fat-free mass index, fat-free mass, muscle mass, and handgrip strength were significantly lower in sarcopenic patients (P < .001).

Background/Objectives: Idiopathic inflammatory myopathies (IIMs) are very rare, chronic, immune-mediated diseases that clinically manifest with myositis symptoms and extramuscular manifestations. The rarity of the disease makes it difficult to conduct double-blind studies, and large population-based studies are needed to improve our knowledge of them. This study aims to analyze the hospitalization rates and trends of IIMs between 2010 and 2024. Additionally, a characterization of the IIM patient population in Poland was conducted. Methods: A retrospective study using data from the Nationwide General Hospital Morbidity Study (NGHMS) between 2010 and 2024 was conducted. Data included demographic characteristics, principal diagnosis, hospitalization, and in-hospital mortality. Classification in accordance with ICD-10 was applied. A total of 7139 patients were included. Appropriate descriptive analyses, comparative tests, Poisson regression for incidence trends, and logistic regression for mortality predictors were performed, with p < 0.05 considered statistically significant. Results: The median IIM incidence in the Polish population was 1.209 per 100,000 per year. The overall female-to-male ratio was 1.68. Hospitalization and mortality trends for IIM were stable over the time period. The incidence among voivodeships in Poland was comparable. Conclusions: Nationwide, comprehensive data are an extremely valuable tool for researching and understanding diseases as rare as IIMs. The incidence, hospitalization, and mortality rates of IIMs in Poland were stable between 2010 and 2024.

Background/Objectives: Anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody is associated with dermatomyositis/clinically amyopathic dermatomyositis and a high risk of rapidly progressive interstitial lung disease (RP-ILD). Methods: We retrospectively analyzed 34 consecutive anti-MDA5-positive patients evaluated at a tertiary Advanced Lung Disease and Lung Transplant center between 1 January 2018 and 31 December 2024. Results: Of 1720 interstitial lung disease (ILD) evaluations, 34 (2.0%) were anti-MDA5 positive (median age 51 years; 56% female). ILD was present in 85%; RP-ILD developed in 13 patients (38%). Overall mortality was 32%, with 11 of 13 deaths occurring in the RP-ILD subgroup (85% mortality). Six patients were listed for lung transplantation and three underwent transplantation; all three remained alive at last follow-up (24-49 months). Right-heart catheterization performed in 11 patients demonstrated pulmonary hypertension (PH) in the majority (64%). Inpatient presentation and elevated ferritin were associated with RP-ILD; elevated CRP and RP-ILD predicted mortality. Conclusions: We conclude that anti-MDA-5-associated ILD carries high mortality when RP-ILD develops. Early recognition, aggressive immunosuppression, systematic consideration of PH, and timely referral for lung transplantation can improve outcomes in selected patients. Routine screening for anti-MDA-5 antibodies should be considered in younger patients with new or progressive ILD.

Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (anti-HMGCR) immune-mediated necrotizing myopathy (IMNM) is a rare autoimmune myopathy that should be suspected in patients with progressive proximal muscle weakness and marked creatine kinase (CK) elevation persisting despite statin discontinuation, although distinguishing it from more common conditions such as post-viral myositis and toxic statin-associated myopathy remains a major diagnostic challenge. We report the case of a 64-year-old man receiving long-term atorvastatin therapy who presented with progressive proximal muscle weakness, dysphagia, and severe hyperCKemia following a self-limited flu-like illness, initially raising post-viral myositis as the leading hypothesis. Despite immediate statin discontinuation and aggressive intravenous hydration, severe hyperCKemia persisted during the first week of supportive management alone, and the neurological deficits did not improve, prompting further investigation and empirical immunosuppression. Muscle magnetic resonance imaging demonstrated multifocal inflammatory muscle edema, while extensive investigation for infectious, metabolic, endocrine, neoplastic, and autoimmune causes was unrevealing. Muscle biopsy showed a necrotizing myopathy with pauci-inflammatory features, and anti-HMGCR antibodies, which had not been included in the initial extended myositis panel and required a dedicated request, were subsequently identified, confirming statin-associated IMNM. Treatment with high-dose corticosteroids followed by methotrexate produced progressive biochemical improvement and complete recovery of muscle strength and swallowing function, with CK falling to 376 U/L (N 30-200 U/L) at the last follow-up (four months after admission). The principal educational value of this case lies in the diagnostic reasoning rather than in the diagnosis itself: it was the failure of the clinical and biochemical course to follow the anticipated trajectory, rather than the initial presentation, that prompted reconsideration of the working hypothesis. Dysphagia, less commonly reported in post-viral myositis and toxic statin-associated myopathy, together with persistent severe hyperCKemia despite statin withdrawal, should prompt evaluation for anti-HMGCR IMNM.

Flagellate erythema is an exceptionally rare cutaneous manifestation of idiopathic inflammatory myopathies (IIM) with poorly understood clinical significance. We report two patients who developed zebra-like flagellate erythema: one with immune-mediated necrotising myopathy (IMNM) and one with anti-Mi-2-positive cancer-associated myositis (CAM). To place these observations in broader context, we performed a thorough literature search across PubMed, Scopus and Embase and identified 34 previously published cases of IIM-associated flagellate erythema. The most frequent underlying diagnosis was dermatomyositis (n = 14, 41.2%), followed by CAM (n = 9, 26.5%), amyopathic/clinically amyopathic dermatomyositis (n = 6, 17.6%), and juvenile dermatomyositis (n = 5, 14.7%). Anti-TIF1-γ antibodies were identified in 6 of 34 patients (17.6%) and anti-MDA5 antibodies in 7 of 34 (20.6%); malignancy was reported in 9 of 34 (26.5%), while a substantial proportion of cases were seronegative or lacked antibody testing. Muscle weakness or myalgia was present in most of the combined cohort, and a fatal outcome was reported in approximately one-fifth of cases. Our cases expand the clinical and serological spectrum of myositis-associated flagellate erythema, including the first report of this presentation in IMNM. While flagellate erythema has most often been reported with CAM or anti-TIF1-γ antibodies, the literature remains inconsistent, and current case-report-based evidence does not support a definitive association with any single serological or clinical subgroup. In our patients, flagellate erythema coincided with disease exacerbation; although this should be interpreted cautiously given the limited evidence, its occurrence may warrant careful evaluation for IIM flare and, where appropriate, malignancy screening.