Systemic auto-inflammatory manifestations in patients with spondyloarthritis - 24/11/24
, Tadej Avčin b, cHighlights |
• | 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. |
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.
Il testo completo di questo articolo è disponibile in 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
Articolo 105772- Dicembre 2024 Ritorno al numero
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