Antisynthetase syndrome (ASS) is a systemic autoimmune disorder classified as a subtype of the idiopathic inflammatory myopathies (IIM). The condition is defined by the presence of mutually exclusive autoantibodies directed against an aminoacyl-tRNA synthetase along with typical clinical manifestations, including myositis, Raynaud's phenomenon, arthritis, skin lesions such as mechanic hands, or interstitial lung disease (ILD). Anti-synthetase syndrome associated interstitial lung disease (ASS-ILD) can range from mild forms to rapidly progressive disease, which may lead to chronic pulmonary damage if misdiagnosed or inadequately treated. Patients with IIM carry an increased risk of developing neoplasms, most commonly adenocarcinoma, but not lymphoma or other hematologic malignancies. However, data on the association between ASS and malignancy remain very limited. We report the case of a patient with anti-PL-12 antisynthetase syndrome who subsequently developed NK/T-cell non-Hodgkin lymphoma, supplemented by a review of the pertinent literature.

Systemic sclerosis is a rare autoimmune connective tissue disease characterized by progressive fibrosis of the skin and internal organs, vasculopathy, and the presence of specific autoantibodies. Despite its low prevalence, systemic sclerosis is associated with high morbidity. Early features often include Raynaud phenomenon, hand edema, and fatigue. Diagnosis requires a comprehensive approach, including clinical assessment, laboratory evaluation, imaging, and pulmonary function testing. The American College of Rheumatology and European Alliance of Associations for Rheumatology (formerly the European League Against Rheumatism) provide classification criteria and updated treatment recommendations. Management focuses on addressing eight disease domains: Raynaud phenomenon, digital ulcers, pulmonary artery hypertension, interstitial lung disease, renal crisis, gastrointestinal involvement, skin fibrosis, and musculoskeletal involvement. Vasodilator therapy is first-line treatment for Raynaud phenomenon, whereas phosphodiesterase-5 inhibitors and intravenous iloprost are used to treat digital ulcers. Combination therapy with phosphodiesterase-5 inhibitors and endothelin receptor antagonists is first-line treatment for pulmonary artery hypertension. Mycophenolate mofetil is the preferred treatment for interstitial lung disease.

Erasmus syndrome is an uncommon condition marked by the onset of systemic sclerosis (SSc) due to extended exposure to silica. In this case, we highlight the significant health risks associated with silica exposure in artisanal mining (galamsey), leading to the development of Erasmus syndrome. We present the case of a 33-year-old male artisanal miner with a 14-year work history who presented with a 2-year history of recurrent cough, difficulty breathing, and fever. He later developed hypopigmentation and skin thickening on his face, neck, torso, and knees, along with a productive cough and low-grade fever. He visited multiple healthcare facilities on account of the above complaints. At one of such visits, he was prescribed antituberculosis (TB) medication even though the sputum for GeneXpert was negative. On examination, he was in respiratory distress with an oxygen saturation of 78% on room air and required oxygen via a nonrebreather mask at 10 L/min, which raised the oxygen saturation to 94%, and had bronchial breath sounds in the right lung zones. Vocal resonance was increased bilaterally in the middle lung zones. He had salt-and-pepper dermopathy with tight and adherent skin on his face, chest, back, and knees. He additionally had microstomia, rat bite sign of the fingers, and Raynaud's phenomenon. Antinuclear antibody by indirect immunofluorescence on hep-2 cells was positive (1:320), and Antitopoisomerase 1 antibodies were also positive. Chest CT findings were consistent with complicated silicosis. The patient was diagnosed with Erasmus syndrome and started on immunosuppressive therapy. We highlight the significant health risks associated with silica exposure in artisanal mining (galamsey), leading to the development of Erasmus syndrome. Preventing silica exposure remains crucial, necessitating stringent workplace safety measures and occupational health practices to protect individuals in high-risk professions.

Infrared thermography (IRT) is an emerging noninvasive imaging modality that provides real-time, contactless assessment of skin surface temperature, reflecting underlying vascular perfusion. This narrative review explores the principles, clinical utility, advantages, limitations, and future potential of IRT in vascular diagnostics and monitoring. IRT has demonstrated diagnostic relevance across a spectrum of vascular conditions, including peripheral arterial disease, diabetic foot complications, venous insufficiency, Raynaud's phenomenon, and postoperative vascular monitoring. Its key benefits - such as radiation-free imaging, portability, and dynamic functional assessment - make it especially valuable for use in vulnerable populations and resource-limited settings. However, challenges such as environmental sensitivity, lack of standardized imaging protocols, and limited specificity necessitate further validation. With the integration of artificial intelligence and wearable technology, IRT holds significant promise as a complementary tool in modern vascular medicine.

Background. Membranous nephropathy (MN) is generally primary, but it can also occur as a secondary form in association with infections, neoplasms or autoimmune diseases. Systemic Sclerosis (SSc), especially in its sine scleroderma forms or in its early stages, rarely manifests itself as MN. Case report. A 60-year-old woman with onset of nephrotic syndrome and histological picture of MN, in the absence of systemic manifestations. The patient subsequently developed episodes of acute renal failure, recurrent proteinuria and clinical-serological signs suggestive of autoimmune connective tissue disease, including increasing ANA titre with anticentromere pattern and onset of Raynaud's phenomenon. The second renal biopsy showed an evolving picture with extensive interstitial fibrosis and severe arteriosclerosis, consistent with a secondary form of MN. The patient was treated with the Ponticelli regimen and subsequently with rituximab, achieving significant clinical remission. In light of the capillaroscopy and autoantibody profile, a diagnosis of very early systemic SSc (sine scleroderma) was made. Discussion. This case highlights how MN can represent an early and atypical manifestation of SSc sine scleroderma, preceding systemic manifestations by years. The negative anti-PLA2R test, the presence of antinuclear autoantibodies and rapid histological progression pointed towards a secondary autoimmune aetiology. Repeated renal biopsy and immunological monitoring proved to be key tools for diagnosis and therapeutic management. Conclusion. MN secondary to SSc sine scleroderma is a rare but important condition that requires attention and a multidisciplinary approach. Early classification as a secondary form allows for targeted therapy and potentially prevents progression to end-stage renal failure.

Systemic sclerosis (SSc) is characterized by fibrosis and vasculopathy affecting the skin and internal organs, leading to multiorgan dysfunction. Injury of microvascular endothelial cells (ECs) in SSc impairs blood flow and causes tissue ischemia, leading to vascular complications such as Raynaud's, digital ulcers, and pulmonary hypertension (PH). PH in SSc presents as group 1 pulmonary arterial hypertension or as group 3 PH related to hypoxia and interstitial lung disease (ILD), both major causes of mortality. Analysis of multiome data from SSc ILD-PH lungs inferred transcription factors regulating EC phenotype, including FOSL2. Overexpression of FOSL2 in transgenic mice (Fosl2tg) leads to vascular changes mirroring human SSc-PH, such as intimal thickening and fibrosis. scRNA-Seq analysis of altered EC gene expression in Fosl2tg mice showed strong overlap with altered EC gene expression in SSc-ILD-PH. Overlapping as well as discrete EC gene expression in Sugen/hypoxia- and hypoxia-treated mice suggested that FOSL2 regulates both hypoxia-dependent and -independent pathways in Fosl2tg mice and SSc-ILD-PH. A deep learning model, ChromBPNet, inferred increased AP-1 binding at base pair resolution in SSc-ILD-PH ECs, and binding to the same motifs was found upon FOSL2 overexpression in primary vascular ECs, highlighting FOSL2's key role in driving the pathological changes seen in SSc-ILD-PH.

Systemic sclerosis (SSc) is a rare autoimmune disease defined by immune dysregulation, vasculopathy, and progressive fibrosis of the skin and internal organs. Despite advances in care, major complications such as interstitial lung disease (ILD) and myocardial involvement remain the leading causes of morbidity and mortality. Current assessment tools, such as the modified Rodnan skin score, pulmonary function tests, and nailfold capillaroscopy, are limited by subjectivity and interobserver variability. Artificial intelligence (AI) is reshaping this landscape. Machine learning, deep learning, and radiomics have shown potential to enhance disease phenotyping, risk stratification, and imaging quantification in SSc. AI-driven high-resolution computed tomography analysis enables automated fibrosis segmentation and radiomic risk modeling in SSc-ILD. Computer vision approaches applied to nailfold capillaroscopy achieve expert-level agreement for microvascular staging, whereas emerging digital tools aim to quantify Raynaud phenomenon dynamics. In skin disease, AI-assisted ultrasonography, optical imaging, and histopathology provide reproducible quantification of dermal remodeling. However, most applications remain exploratory, based on small or single-center data sets with limited external validation. Challenges such as data heterogeneity, annotation burden, and the "small data" constraints of rare diseases limit immediate clinical translation. This narrative review critically examines current AI applications in SSc, highlighting methodologic limitations, translational readiness, and future directions toward robust, multicenter, and ethically governed implementation.

Differentiating reactive lymphadenopathies in the context of autoimmune disease from Idiopathic Multicentric Castleman Disease (iMCD) poses a significant diagnostic challenge. Castleman-like histological features have been described in various autoimmune disorders, necessitating a strict and comprehensive integration of clinical and laboratory findings to reach the correct diagnosis. Although the Castleman Disease Collaborative Network (CDCN) consensus guidelines list several autoimmune conditions as exclusion criteria for an iMCD diagnosis, mixed connective tissue disease (MCTD) is not currently among them. We report the case of a 77-year-old woman presenting with fatigue, Raynaud's phenomenon, sclerodactyly, mild generalized lymphadenopathy, in whom the lymph node biopsy revealed a Castleman-like histology. The absence of systemic inflammatory symptoms and the presence of high-titer anti-U1- RNP antibodies were, however, inconsistent with iMCD, favouring the diagnosis of a reactive Castleman-like lymphadenitis secondary to MCTD. This report highlights that Castleman-like lymphadenopathy can occur in MCTD, closely mimicking iMCD. Therefore, in patients with autoimmune diseases not explicitly listed among the CDCN exclusion criteria, comprehensive clinicopathological integration is essential to avoid misdiagnosis and potentially inappropriate antiIL-6-based therapy.

Systemic sclerosis (SSc) is a complex systemic autoimmune rheumatic disease with marked clinical heterogeneity. Cutaneous manifestations affecting the hands and wrists include vascular insufficiency, early edema (puffy fingers), followed by progressive skin fibrosis and atrophy (sclerodactyly). This progressive skin tightening results in joint stiffness, deformity, functional impairment and reduced quality of life. Beyond skin changes, hand involvement may also include inflammatory arthritis, joint contractures, tendon friction rubs, Raynaud phenomenon, digital ulcers, acro-osteolysis, and calcinosis, all of which can further impair hand function, significantly affecting individuals' ability to perform routine occupational and daily tasks requiring grasping, gripping, and fine motor dexterity. In this article, we synthesize the evidence evaluating manual therapy, prescribed hand exercises, self-administered hand exercise protocols, telerehabilitation, paraffin wax, therapeutic ultrasound, manual lymphatic drainage, and dynamic splinting to improve hand function in people with SSc.