BACKGROUND:Systemic lupus erythematosus (SLE) leads to renal lesions, which may be clinically silent in patients with little or no proteinuria. Early detection of these lesions may improve prognosis, but early markers are controversial. This study ai...

Purpose: This study aimed to assess liver involvement and investigate its correlation with rapidly progressive interstitial lung disease (RP-ILD) and mortality in anti-melanoma differentiation-associated gene 5 antibody-positive (anti-MDA5 positive) DM patients. Patients and Methods: This retrospective study included 159 patients diagnosed with anti-MDA5 positive DM or anti-synthetase syndrome (ASyS). Clinical features and laboratory findings were compared between patients with anti-MDA5 positive DM and patients with ASyS. In the anti-MDA5 positive DM cohort, clinical features and laboratory findings between patients with liver involvement and without liver involvement were further compared. The effects of liver involvement on the overall survival (OS) and development of RP-ILD were also analyzed using Kaplan–Meier method and Cox regression analysis. Results: Levels of serum aspartate aminotransferase (AST), alanine transaminase (ALT), γ-glutamyl transferase (γGT) and alkaline phosphatase (ALP) were all significantly higher in patients with anti-MDA5 positive DM than those in patients with ASyS. In our cohort of anti-MDA5 positive DM patents, 31 patients (34.4%) were complicated with liver involvement. Survival analysis revealed that serum ferritin > 1030.0 ng/mL (p< 0.001), ALT > 103.0 U/l (p< 0.001), AST > 49.0 U/l (p< 0.001), γGT > 82.0 U/l (p< 0.001), ALP > 133.0 U/l (p< 0.001), lactate dehydrogenase (LDH)> 474.0 U/l (p< 0.001), plasma albumin (ALB) < 35.7 g/l (p< 0.001) and direct bilirubin (DBIL) > 2.80 μmol/l (p=0.002) predicted poor prognosis. The incidence of RP-ILD increased remarkably in patients with liver involvement compared to patients without liver involvement (58.1% vs 22.0%, p=0.001). Multivariate analysis revealed that elevated serum ALT level was an independent risk factor for mortality (HR 6.0, 95% CI 2.3, 16.2, p< 0.001) and RP-ILD (HR 5.9, 95% CI 2.2, 15.9, p< 0.001) in anti-MDA5 positive DM patents. Conclusion: Liver involvement is common in patients with anti-MDA5 positive DM. Elevated serum ALT level was an independent risk factor for RP-ILD and mortality in patients with anti-MDA5 positive DM.

Background: Systemic lupus erythematosus (SLE) is a systemic autoimmune disease involving multiple organs throughout the body. The health care–seeking behaviors, disease progression of SLE, and patients' knowledge of and attitudes toward SLE have not been characterized in China. Objective: The aim of this study was to depict the health care–seeking behaviors, disease progression, and medications in patients with SLE and to examine the factors associated with their disease flares, knowledge, and attitudes toward SLE in China. Methods: We conducted a cross-sectional survey in 27 provinces in China. Descriptive statistical methods were used to depict the demographic characteristics, health care–seeking behaviors, medications, and health status. Multivariable logistic regression models were used to identify the factors associated with disease flares, medication changes, and attitudes toward SLE. An ordinal regression model was used to examine the factors associated with the knowledge of the treatment guidelines. Results: We recruited 1509 patients with SLE, and 715 had lupus nephritis (LN). Approximately 39.96% (603/1509) of the patients with SLE were primarily diagnosed with LN, and 12.4% (112/906) developed LN (mean time 5.2 years) from non-LN. Patients whose registered permanent residences or workplaces in other cities from the same province and adjacent provinces seeking health care accounted for 66.9% (569/850) and 48.8% (479/981) of the patients with SLE in the provincial capital cities, respectively. Mycophenolate mofetil was the most commonly used immunosuppressive drug in patients without LN (185/794, 23.3%) and patients with LN (307/715, 42.9%). Femoral head necrosis (71/228, 31.1%) and hypertension (99/229, 43.2%) were the most common adverse event (AE) and chronic disease during treatment, respectively. Change of hospitals for medical consultation (odds ratio [OR] 1.90, 95% CI 1.24-2.90) and development of 1 chronic disease (OR 3.60, 95% CI 2.04-6.24) and AE (OR 2.06, 95% CI 1.46-2.92) and more were associated with disease flares. A pregnancy plan (OR 1.58, 95% CI 1.18-2.13) was associated with changes in medication. Only 242 (16.03%) patients with SLE were familiar with the treatment guidelines, and patients with LN tended to be more familiar with the disease (OR 2.20, 95% CI 1.81-2.68). After receiving treatment, 891 (59.04%) patients changed their attitudes toward SLE from fear to acceptance, and patients with college education or higher (OR 2.09, 95% CI 1.10-4.04) were associated with a positive attitude toward SLE. Conclusions: A large proportion of patients seeking health care in the provincial capital cities of China migrated from other cities. Persistent monitoring of potential AEs and chronic diseases during SLE treatment and managing patients who changed hospitals for medical consultation are essential for controlling disease flares. Patients had insufficient knowledge about SLE treatment guidelines and would benefit from health education to maintain a positive attitude toward SLE.

Axial gout is an underrecognized manifestation of monosodium urate (MSU) crystal deposition and frequently mimics inflammatory, infectious, or mechanical spinal disorders, particularly without overt hyperuricaemia. We report a 29-year-old man with recurrent low back pain for over two years and recurrent nephrolithiasis who had undergone three extracorporeal shock wave lithotripsy sessions with only transient relief. Admission laboratory investigations showed normal serum urate (262 µmol/L, ~ 4.4 mg/dL) but markedly elevated C‑reactive protein (119 mg/L) and erythrocyte sedimentation rate (53 mm/h). Retrospective review of prior hospitalizations for ureteral stones revealed that routine serum urate measurements had never been elevated. Conventional imaging, including plain radiography and non‑contrast computed tomography, was inconclusive. Dual‑energy computed tomography (DECT) revealed MSU deposition in the L4/L5 and L5/S1 facet joints and sacral foramina, supporting the diagnosis of axial gout. The patient was treated with etoricoxib (60 mg daily) and febuxostat (20 mg daily). On telephone follow‑up on 27 May 2026 (the only follow‑up to date), he reported that the J stent had been removed and low back pain had completely resolved without recurrence. A single repeat laboratory test performed locally was reported as normal, though specific values were unavailable. A systematic literature review of 58 eligible articles revealed that the lumbar spine was most frequently involved (≈ 60%), DECT sensitivity ranged from 78% to 100%, and 77.5% of historically reported cases required surgical diagnosis, underscoring the underrecognition of axial gout in non-surgical settings. This case‑based review highlights that axial gout should be considered in young non‑hyperuricaemic patients with persistent axial pain, and that DECT is a valuable non‑invasive diagnostic tool when interpreted alongside clinical and laboratory features.

Introduction: Dermatomyositis (DM), particularly the anti-TIF1γ‑positive subtype, is associated with a substantially elevated risk of malignancy. However, the peripheral immune landscape of this autoantibody-defined subset, especially in patients without concurrent cancer, remains incompletely understood. Methods: We performed single‑cell RNA sequencing of peripheral blood mononuclear cells (PBMCs) from five patients with anti‑TIF1γ‑positive DM (the subtype with the highest malignancy risk) and compared their profiles with those of two other DM subtypes: anti‑MDA5‑positive DM (intermediate malignancy risk) and anti‑synthetase syndrome (ASyS, low malignancy risk). Results: All three subtypes exhibited profound immune remodeling, characterized by reduced frequencies of cytotoxic lineage cells. Anti‑TIF1γ‑positive DM showed a compensatory shift toward humoral immunity, with increased B‑lineage cells, whereas classical monocyte elevation was specific to the anti‑MDA5‑positive DM and ASyS subtypes. Notably, anti‑TIF1γ‑positive DM was uniquely distinguished by an increased frequency of immunosuppressive regulatory T (Treg) cells, a finding validated by immunohistochemistry in patient skin tissue. Conclusion: These results delineate a peripheral immunological signature of anti‑TIF1γ‑positive DM, marked by compromised cytotoxic surveillance, aberrant humoral responses, and selective Treg enrichment. We hypothesize that this Treg elevation may contribute to an immunosuppressive milieu that favors malignancy, although this mechanism remains speculative and requires functional validation in prospective cohorts.

Background: Spontaneous pneumomediastinum (SPNM) occurred more frequently in dermatomyositis (DM) patients with anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody than in DM patients without anti-MDA5 antibody. SPNM has been indicated as a risk factor for death in anti-MDA5-positive DM. There have been no clinical studies evaluating the treatment strategy for SPNM in anti-MDA5-positive DM.Methods: We present a series of five cases in which the administration of Janus kinase (JAK) inhibitors improved the clinical condition of anti-MDA5-positive DM patients with SPNM.Results: Clinical conditions of the four patients who were given JAK inhibitors after the diagnosis of SPNM were all improved. SPNM symptoms of the patient who was not commenced on JAK inhibitors progressed, and the patient died of severe pulmonary infection.Conclusion: JAK inhibitors appear to be an efficient therapeutic option in the treatment of anti-MDA5-positive DM with SPNM.