Class 1. Pulmonary Hypertension Associated with Connective Tissue Disease

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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Pulmonary manifestations of juvenile vs. adult systemic sclerosis: insights into pathophysiological and clinical features

Gabriele Di Pasquale, Nicholas Caione, Alessio Di Berardino, Giulio Di DonatoUniversity of L’Aquila.Italy Pediatric PulmonologyPediatr Pulmonol 2024; DOI: 10.1002/ppul.27347 AbstractJuvenile systemic sclerosis (jSSc), the pediatric counterpart of systemic sclerosis (SSc), is a rare autoimmune disorder characterized by vasculopathy and fibrotic disorders. It ranks among the rheumatologic diseases with the highest rates of morbidity and mortality, predominantly

Pulmonary manifestations of juvenile vs. adult systemic sclerosis: insights into pathophysiological and clinical features Read More »

The role of immune cells in the pathogenesis of connective tissue diseases-associated pulmonary arterial hypertension

Zhe Li, Juan Ma, Xuejing Wang, Liquan Zhu, Yu Gan, Baoquan DaiWeifang Maternal and Child Health Hospital. Shandong Second Medical University.China Frontiers in ImmunologyFront Immunol 2024; 15: DOI: 10.3389/fimmu.2024.1464762 AbstractConnective tissue diseases-related pulmonary arterial hypertension (CTD-PAH) is a disease characterized by an elevated pulmonary artery pressure that arises as a complication of connective tissue diseases. The

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Utility of factor D and other alternative complement factors as biomarkers in systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH)

Eva Petrow, Changyong Feng, Ashley Frazer-Abel, Roberta Goncalves Marangoni, Katie Lutz, William C. Nichols, V. Michael Holers, Christopher Ritchlin, R. James White III, Benjamin D. KormanUniversity of Rochester Medical Center. University of Colorado School of Medicine. Cincinnati Children’s Medical Center and University of Cincinnati College of Medicine. United States Seminars in Arthritis and RheumatismSemin Arthritis

Utility of factor D and other alternative complement factors as biomarkers in systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) Read More »

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