Class 1. Pulmonary Hypertension Associated with Connective Tissue Disease

Echocardiographic assessment of pediatric lupus nephritis and its correlation with disease activity in a tertiary care hospital

Afroza Begum, Amina Akter, Nadira Sultana, Abdullah Al Mamun, Tahmina Jesmin, Syed Saimul Huque, Shanjida Sharmim, Sharmim Akter Luna, Shamsunnahar Shanta, Samina Masud Santa, Sabina Sultana, Ranjit RoyBangabandhu Sheikh Mujib Medical University. Gonoshasthaya Samaj Vittik Medical College. Evercare Hospital.Bangladesh LupusLupus 2025; DOI: 10.1177/09612033251367175 AbstractSystemic lupus erythematosus (SLE) is a complex autoimmune disease with multisystem involvement. Lupus […]

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Systemic Sclerosis: A Key Model of Endothelial Dysfunction

Vincenzo Zaccone, Lorenzo Falsetti, Silvia Contegiacomo, Serena Cataldi, Devis Benfaremo, Gianluca MoronciniMarche Polytechnic University.Italy BiomedicinesBiomedicines 2025; 13: DOI: 10.3390/biomedicines13071771 AbstractSystemic sclerosis (SSc) is a heterogeneous disease characterized by vascular alterations, immune dysregulation, and fibrosis. Solid evidence supports the hypothesis that endothelial dysfunction is the key player in SSc vascular injury and a critical factor concurring to

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Utility of patient-reported outcomes for pulmonary symptoms and sleep disturbance and impairment in children with systemic juvenile idiopathic arthritis

Kim Nguyen, Shima Yasin, Ndate Fall, Alexei A. Grom, Hermine I. Brunner, Christopher Towe, Grant S. SchulertTriHealth Hospital System. University of Cincinnati College of Medicine and Cincinnati Children’s Hospital Medical Center.United States Pediatric Rheumatology Online JournalPediatr Rheumatol Online J 2025; 23: DOI: 10.1186/s12969-025-01126-y AbstractBackground: Patient-reported outcomes (PROs) are critical assessment tools for clinical practice, observational studies, and

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Juvenile Systemic Sclerosis Presenting With Severe Pulmonary Vascular Disease: ECMO Stabilization During Aggressive Therapy Resulting in a Favorable Outcome

Stephanie M. Tsoi, Shannon Cheung, Roberto Alejandro Valdovinos, Elena Amin, Hythem Nawaytou, Elizabeth Colglazier, Claire Parker, April Edwell, Susan Kim, Jeffrey R. FinemanUniversity of California San Francisco. Pulmonary CirculationPulm Circ 2025; 15: DOI: 10.1002/pul2.70106 AbstractJuvenile systemic sclerosis (jSSc) associated pulmonary hypertension (PH) is rare, but, the leading cause of morbidity and mortality in jSSc. This is

Juvenile Systemic Sclerosis Presenting With Severe Pulmonary Vascular Disease: ECMO Stabilization During Aggressive Therapy Resulting in a Favorable Outcome Read More »

Evaluating the relevance of the 2024 BSR systemic sclerosis guideline to juvenile systemic sclerosis

Clare E. Pain, Hanna Lythgoe, Emily Willins, Sunil Sampath, Samundeeswari Deepak, Kathryn S. Torok, Phuoc H. Duong, Shahin Moledina, Juliana Silva, Clarissa Pilkington, Eslam Al-Abadi, Christopher P. DentonAlder Hey Children’s Hospital. Royal Manchester Children’s Hospital. Great North Children’s Hospital. Nottingham Children’s Hospital. UPMC Children’s Hospital of Pittsburgh. Birmingham Women’s and Children’s Hospital. University College London.

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Successful control of recurrent MAS by canakinumab in a Sjogren syndrome patient with homozygous MEFV P369S variants, and review of literatures

Noboyuki Ono, Motoki Yoshimura, Toshiya Nishida, Yusuke Yamauchi, Goro Doi, Yoko Fuyuno, Motoshi Sonoda, Hiroaki NiiroFukuoka City Hospital, Kyushu University Hospital, Beppu Hospital, and Kyushu University. Iizuka Hospital. Japan Modern Rheumatology Case ReportsMod Rheumatol Case Rep 2025; DOI: 10.1093/mrcr/rxaf016 AbstractMacrophage activation syndrome (MAS) is an autoinflammatory condition, which severely complicates autoimmune diseases, such as SJIA, AOSD

Successful control of recurrent MAS by canakinumab in a Sjogren syndrome patient with homozygous MEFV P369S variants, and review of literatures Read More »

Anti-fibroblast and anti-endothelial cell autoantibodies in pulmonary arterial hypertension (PAH) in patients with connective tissue diseases (CTD)

Benjamin Thoreau, Arthur Ranaud, Philippe Chafey, Guilhem Clary, Morgane La Gall, Cédric Broussard, Odile Launay, David Launay, Eric Hachulla, Christophe Deligny, Alban-Elouen Baruteau, Anaïs Vallet-Pichard, Benjamin Chaigne, Azzeddine Yaici, Oliver Sitbon, David Montani, Marc Humbert, Luc MouthonUniversité Paris Cité. Hôpital Cochin and Assistance Publique-Hôpitaux de Pari. de l’Est et de l’Ouest. Hôpital Pierre Zobda-Quitman. Nantes

Anti-fibroblast and anti-endothelial cell autoantibodies in pulmonary arterial hypertension (PAH) in patients with connective tissue diseases (CTD) Read More »

The clinical assessment of lung involvement in patients with Still’s disease, results from the multicentre international AIDA Network Still’s Disease Registry

Piero Ruscitti, Antonio Vitale, Ilenia Di Cola, Valeria Caggiano, Pierpaolo Palumbo, Ernesto Di Cesare, Andrea Hinojosa-Azaola, Jiram Torres-Ruiz, Guillermo Arturo, Guaracha-Basañez, Eduardo Martín-Nares, Giuseppe Lopalco, Maria Morrone, Florenzo Iannone, Henrique A. Mayrink Giardini, Rafael Alves Cordeiro, Isabele, Parente de Brito Antonelli, Onorina Berardicurti, Luca Navarini, Francesco Ciccia, Maria Chiara Visconti, Daniela Iacono, Haner Direskeneli, Sukran

The clinical assessment of lung involvement in patients with Still’s disease, results from the multicentre international AIDA Network Still’s Disease Registry Read More »

Pulmonary Arterial Hypertension Incidence in Scleroderma Patients Treated with Bosentan for Digital Ulcers: Evidence from the Italian SPRING Registry

Fabio Cacciapaglia, Rossella De Angelis, Clodoveo Ferri, Gianluigi Bajocchi, Silvia Bellando-Randone, Cosimo Bruni, Martina Orlandi, Marco Fornaro, Edoardo Cipolletta, Giovanni Zanframundo, Roberta Foti, Giovanna Cuomo, Alarico Ariani, Edoardo Rosato, Gemma Lepri, Francesco Girelli, Elisabetta Zanatta, Silvia Laura Bosello, Ilaria Cavazzana, Francesca Ingegnoli, Maria De Santis, Giuseppe Murdaca, Giuseppina Abignano, Pettiti Giorgio, Alessandra Della Rossa, Maurizio Caminiti, Annamaria Iuliano, Giovanni Ciano, Lorenzo Beretta, Gianluca Bagnato, Ennio Lubrano, Ilenia De Andres, Alessandro Giollo, Marta Saracco, Cecilia Agnes, Corrado Campochiaro, Federica Lumetti, Amelia Spinella, Luca Magnani, Giacomo De Luca, Veronica Codullo, Elisa Visalli, Carlo Iandoli, Antonietta Gigante, Greta Pellegrino, Franco Cozzi, Maria Grazia Lazzaroni, Elena

Pulmonary Arterial Hypertension Incidence in Scleroderma Patients Treated with Bosentan for Digital Ulcers: Evidence from the Italian SPRING Registry Read More »

Diagnostic enigma of pulmonary hypertension: Tale from a series of three patients

Anil Kumar Singhi, Anup Banerji, Nandini Biswas, Soumen DasMedica Super Specialty Hospital. India Medical Journal Armed Forces IndiaMed J Armed Forces Ind 2024; 80 (Suppl 1): S320-S324DOI: 10.1016/j.mjafi.2023.08.003 AbstractPulmonary hypertension (PH) is a complex condition with multiple etiological factors. The ability to identify a potential underlying cause is crucial for accurate diagnosis, patient management, and prognostication.

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