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Systemic auto-inflammatory manifestations in patients with spondyloarthritis - 24/11/24

Doi : 10.1016/j.jbspin.2024.105772 
Carla Gaggiano a, Mojca Zajc Avramovič b, c, Antonio Vitale a, Nina Emeršič b, c, Jurgen Sota a, Nataša Toplak b, c, Stefano Gentileschi a, Valeria Caggiano a, Maria Tarsia d, Gašper Markelj b, c, Tina Vesel Tajnšek b, c, Claudia Fabiani e, Anja Koren Jeverica b, Bruno Frediani a, Maria Antonietta Mazzei f, Luca Cantarini a, , Tadej Avčin b, c
a Rheumatology Unit, Department of Medical Sciences, Surgery and Neuroscience, University of Siena and Azienda Ospedaliero-Universitaria Senese, Viale Mario Bracci 16, 53100 Siena, Italy 
b Department of Allergology, Rheumatology and Clinical Immunology, University Children's Hospital, University of Ljubljana and University Medical Centre Ljubljana, Bohoričeva ulica 20, 1000 Ljubljana, Slovenia 
c Department of Pediatrics, Faculty of Medicine, University of Ljubljana, Bohoričeva ulica 20, 1000 Ljubljana, Slovenia 
d Clinical Pediatrics, Department of Molecular Medicine and Development, University of Siena and Azienda Ospedaliero-Universitaria Senese, Viale Mario Bracci 16, 53100 Siena, Italy 
e Ophthalmology Unit, Department of Medical Sciences, Surgery and Neuroscience, University of Siena and Azienda Ospedaliero-Universitaria Senese, Viale Mario Bracci 16, 53100 Siena, Italy 
f Unit of Diagnostic Imaging, Department of Medical Sciences, Surgery and Neuroscience, University of Siena and Azienda Ospedaliero-Universitaria Senese, Viale Mario Bracci 16, 53100 Siena, Italy 

Corresponding author. Research Center of Systemic Auto-inflammatory Diseases and Behçet's Disease Clinics, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Rheumatology Unit, Policlinico “Le Scotte”, viale Mario Bracci 16, 53100 Siena, Italy.Research Center of Systemic Auto-inflammatory Diseases and Behçet's Disease Clinics, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Rheumatology Unit, Policlinico “Le Scotte”viale Mario Bracci 16Siena53100Italy

Highlights

Spondyloarthritis has been associated with several auto-inflammatory conditions, including familial Mediterranean fever, Behçet's disease, undifferentiated systemic auto-inflammatory disease and, more recently, Still's disease.
This research offers a detailed characterization of a cohort diagnosed with “systemic spondyloarthritis” leveraging patients with non-systemic spondyloarthritis and Still's disease as benchmarks for comparing articular and systemic presentations.
A possible relationship between Still's disease and spondyloarthritis has been suggested, based on the analysis of new cases and those reported in the literature, hypothesizing a role for uncontrolled inflammation in the shift towards the spondyloarthritis phenotype.
These findings advocate for the inclusion of sacroiliitis in the diagnostic work-up of pediatric and adult patients exhibiting unexplained systemic auto-inflammatory manifestations. Also, this study prompts further investigative efforts aimed to elucidate the pathophysiological mechanisms connecting the systemic and axial manifestations of systemic spondyloarthritis.

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Abstract

Objectives

(1) characterizing a group of spondyloarthritis (SpA) patients with systemic auto-inflammatory symptoms (S-SpA); (2) comparing SpA features with and without auto-inflammatory symptoms; (3) comparing the auto-inflammatory features of S-SpA and Still's disease (SD).

Methods

Retrospective observational study. Clinical data of adult and pediatric patients with S-SpA, SD or SpA were collected retrospectively and analyzed.

Results

Forty-one subjects with S-SpA, 39 with SD and 42 with SpA were enrolled. The median latency between systemic and articular manifestations in S-SpA was 4.4 (IQR: 7.2) years. S-SpA and SpA had similar frequency of peripheral arthritis and enthesitis (N.S.), while tenosynovitis was more frequent (P=0.01) and uveitis less frequent (P<0.01) in S-SpA. MRI showed signs of sacroiliac inflammation and damage in both S-SpA and SpA equally (N.S.). S-SpA patients had less corner inflammatory lesions (P<0.05) and inflammation at the facet joints (P<0.01), more interspinous enthesitis (P=0.01) and inter-apophyseal capsulitis (P<0.01). Compared to SD, S-SpA patients had lower-grade fever (P<0.01), less rash (P<0.01) and weight loss (P<0.05), but more pharyngitis (P<0.01), gastrointestinal symptoms (P<0.01) and chest pain (P<0.05). ESR, CRP, WBC, ANC, LDH tested higher in SD (P<0.01). Resolution of systemic symptoms was less frequent in S-SpA than SD on corticosteroid (P<0.01) and methotrexate (P<0.05) treatment. When considering all SD patients, a complete response to corticosteroids in the systemic phase significantly reduced the likelihood of developing SpA (OR=0.06, coefficient −2.87 [CI: −5.0 to −0.8]).

Conclusions

SpA should be actively investigated in patients with auto-inflammatory manifestations, including undifferentiated auto-inflammatory disease and SD.

Le texte complet de cet article est disponible en PDF.

Keywords : Spondyloarthritis, Febrile spondyloarthritis, Still's disease, Systemic juvenile idiopathic arthritis, Adult-onset Still's disease, Auto-inflammatory diseases


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Vol 91 - N° 6

Article 105772- décembre 2024 Retour au numéro
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