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Autoinflammatory & rare systemic diseases

Adult-onset Still's disease, periodic fever syndromes, other monogenic and acquired autoinflammatory disease, IgG4-related disease, relapsing polychondritis, sarcoidosis with joint involvement and other rare systemic inflammatory disease

CurrentRheuma holds 305 Briefs in Autoinflammatory & rare systemic diseases, most often cohort studies and mechanistic studies; the journals with the most are Rheumatology (Oxford), Clin Exp Rheumatol and Ann Rheum Dis.

305 Briefs placed here35 more Briefs indexed under this topic as a secondary topic

Briefs in Autoinflammatory & rare systemic diseases

Newest publication first

  1. Rheumatology (Oxford)

    Development and validation of a text-mining-based algorithm for detection of rare hereditary periodic fever syndromes

    Diagnostic or prognostic study

  2. Lancet Rheumatol

    The IgG4ward! Foundation guidelines on the treatment of IgG4-related disease

    Guideline or statement

  3. RMD Open

    Clinical characteristics, inflammatory biomarkers and genetic background of patients with non-hereditary periodic fever syndromes

    Cohort study

  4. Clin Exp Rheumatol

    Exploring the faecal miRNA profile in familial Mediterranean fever patients and the role of miR-21-5p in inflammatory pathways associated with FMF pathogenesis

    Case-control study

  5. Clin Exp Rheumatol

    Serum ferritin predicts response to tocilizumab in glucocorticoid-resistant adult-onset Still's disease: a prospective real-world cohort study

    Cohort study

  6. Arthritis Rheumatol

    Treatment Approaches to Macrophage Activation Syndrome in Real Life and Priorities for Future Research: Results From the International METAPHOR Project

    Cross-sectional study

  7. Rheumatology (Oxford)

    Beyond NLRP3: expanding the spectrum of NLR-associated autoinflammatory diseases

    Cohort study

  8. Arthritis Rheumatol

    Human Monocytic Models Reveal Genotype‐Dependent Inflammatory Programs in VEXAS Syndrome

    Mechanistic study

  9. RMD Open

    Comparative evaluation of disease activity measures for adult-onset Still’s disease: a multicentre, retrospective, cohort study

    Cohort study

  10. Ann Rheum Dis

    Validation of systemic Juvenile Arthritis Disease Activity Score 10 in adults with Still’s disease

    Diagnostic or prognostic study

  11. Arthritis Rheumatol

    Persistent Interleukin‐18 Fuels Expansion of CD38 + HLA ‐ DR + CD8 + T Cells in Still Disease and Macrophage Activation Syndrome

    Mechanistic study

  12. Rheumatology (Oxford)

    Diagnostic performance of HScore for macrophage activation syndrome complicating Still’s disease in adults: a multicentre case–control study

    Diagnostic or prognostic study

  13. Semin Arthritis Rheum

    Biologic–biologic and biologic–JAK inhibitor combination therapy in refractory systemic autoinflammatory diseases

    Cohort study

  14. Rheumatology (Oxford)

    Fractional exhaled nitric oxide is a non-invasive disease activity marker of IgG4-related disease

    Cross-sectional study

  15. Rheumatology (Oxford)

    Early longitudinal proteomic changes during sirolimus therapy in tocilizumab-refractory idiopathic multicentric Castleman disease

    Mechanistic study

  16. Rheumatology (Oxford)

    Extrafollicular Tph2-like cell dominant tissue pathotype is associated with relapse in IgG4-related disease

    Cohort study

  17. Rheumatology (Oxford)

    Treatment strategies for relapsing IgG4-RD: 12-year experience from a prospective cohort

    Cohort study

  18. Arthritis Rheumatol

    Single‐Cell Profiling of Lymphoid and Myeloid Compartments Reveals Coordinated Immune Dysregulation and Enhanced Monocyte‐Centric Intercellular Communication in Relapsing Polychondritis

    Mechanistic study

  19. Rheumatology (Oxford)

    Association of Th2-like inflammation with Tfh/Tph–germinal center immune programmes in submandibular gland lesions of IgG4-related disease

    Mechanistic study

  20. Arthritis Rheumatol

    Redefining the Landscape: Acquired Mutations Driving Systemic Autoinflammatory Diseases

    Cohort study

  21. Semin Arthritis Rheum

    Disentangling the hepatic risks of familial Mediterranean Fever and colchicine: A propensity score-matched analysis of 19,231 patients

    Cohort study

  22. Ann Rheum Dis

    A phase 2 trial of rilzabrutinib in IgG4-related disease

    Research article

  23. Arthritis Res Ther

    Effects of first-line biologic immunomodulation compared with non-biologic therapies in adult-onset Still’s disease: a two-center cohort study

    Cohort study

  24. Rheumatology (Oxford)

    Lung disease in adult-onset Still’s disease: a Chinese cohort study

    Cohort study

  25. Arthritis Rheumatol

    A Multicenter Integrative Cohort Characterizing the Genetic, Clinical, and Transcriptomic Features of ACP5 Deficiency

    Cohort study

  26. Ann Rheum Dis

    Zanubrutinib monotherapy for IgG4-related head and neck disease

    Research article

  27. Arthritis Care Res (Hoboken)

    Development of a Disease Activity Index for the Assessment of VEXAS Syndrome (VEXAS‐DAI)

    Guideline or statement

  28. Pediatr Rheumatol Online J

    Surgical versus medical management of PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis): a systematic review and network meta-analysis

    Meta-analysis

  29. Rheumatology (Oxford)

    Effectiveness and probability of full disease control with canakinumab in familial Mediterranean fever: real-world data from the AIDA Network

    Cohort study

  30. Rheumatology (Oxford)

    Free IL-18 in NLRC4-associated autoinflammatory disease without macrophage activation syndrome

    Mechanistic study

  31. Arthritis Res Ther

    Evaluating clinical heterogeneity in relapsing polychondritis through unsupervised cluster analysis

    Cohort study

  32. Pediatr Rheumatol Online J

    Spectrum of MEFV variants in 8,252 patients from Eastern and Southeastern Anatolia: a large single-center cohort study (2019–2026)

    Cohort study

  33. RMD Open

    Development and assessment of an assisted diagnosis model using machine learning for identifying adult-onset Still’s disease in fever of unknown origin: a retrospective study in China

    Diagnostic or prognostic study

  34. Rheumatology (Oxford)

    Development and validation of a relapsing polychondritis disease-specific quality of life instrument (ERN ReCONNET RP-QoL)

    Diagnostic or prognostic study

  35. Semin Arthritis Rheum

    Long-term remission and monocyclic course in Still’s disease patients starting canakinumab early: data from the international AIDA network registry

    Cohort study

  36. Scand J Rheumatol

    Clinical and histopathological features of a population-based cohort of 95 consecutive patients with immunoglobulin G4-related disease in Stockholm, Sweden, during a five-year period

    Cohort study

  37. Ann Rheum Dis

    Longitudinal profiling reveals immune dynamics and distinct plasma cell signatures during B-cell depletion in IgG4-related disease

    Mechanistic study

  38. Rheumatology (Oxford)

    Association between biologic exposure and relapse incidence in relapsing polychondritis: a retrospective cohort study

    Cohort study

  39. Joint Bone Spine

    Club Rhumatismes et Inflammation guidelines for anti-interleukin-1 therapy: 2026 update

    Guideline or statement

  40. Semin Arthritis Rheum

    Long-term rheumatologic comorbidities in familial Mediterranean fever

    Cohort study

All 305 Briefs in ExploreShowing the 40 most recently published