Sjögren Syndrome and Inflammatory Myopathies

The answer first

Sjögren syndrome and the inflammatory myopathies (dermatomyositis, polymyositis, immune-mediated necrotising myopathy, inclusion body myositis, antisynthetase syndrome, overlap myositis) are systemic autoimmune diseases with distinctive clinical, serological and prognostic profiles, and they account for a substantial share of long-case and short-case examinations because they integrate rheumatology with pulmonary, neurological, dermatological, and oncological medicine. Sjögren syndrome is characterised by sicca symptoms (dry eyes, dry mouth), anti-Ro/SSA and anti-La/SSB antibodies, and extraglandular manifestations (arthralgia, fatigue, interstitial lung disease, vasculitis, lymphoma). The inflammatory myopathies present with proximal muscle weakness, characteristic rashes (dermatomyositis), and a myositis-specific antibody pattern that defines the clinical subgroup and predicts complications (interstitial lung disease in antisynthetase syndrome; malignancy in dermatomyositis; severe necrotising myopathy in anti-SRP and anti-HMGCR disease). ^{[1] [2]}

The FRACP candidate must be able to defend three positions without hedging: (1) Sjögren syndrome is diagnosed by the 2016 ACR/EULAR criteria — a weighted score (4 or more points) incorporating anti-Ro/SSA antibodies, ocular staining score, Schirmer test, salivary flow rate, and minor salivary gland biopsy, in which anti-La/SSB without anti-Ro does not score — and management is symptomatic (topical therapy, pilocarpine) for sicca symptoms plus systemic immunosuppression for severe extraglandular disease; (2) dermatomyositis carries a substantial cancer risk (occult malignancy in 15 to 25 percent within three years of diagnosis) and mandates a structured malignancy work-up; and (3) the inflammatory myopathies are grouped by their myositis-specific antibodies, and the antibody pattern (anti-Jo-1, anti-Mi-2, anti-TIF1-γ, anti-NXP2, anti-MDA5, anti-SAE, anti-HMGCR, anti-SRP, anti-cN1A) dictates the clinical syndrome, malignancy risk, lung disease risk, and the choice of therapy. Lead with the decision, then the evidence, then the trap. ^{[1] [2] [9]}

References11ShowHide

1. [1]Shiboski CH, Shiboski SC, Seror R, et al. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren's Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts Arthritis Rheumatol, 2017.PMID 27785888
2. [2]Lundberg IE, Tjärnlund A, Bottai M, et al. 2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups Ann Rheum Dis, 2017.PMID 29079590
3. [4]Buonocore TM, Marino S, Albi G, Sakellariou G, et al. Ontology-Enriched Guidelines Retrieval for Complex Rheumatological Cases Stud Health Technol Inform, 2026.PMID 42175019
4. [5]Talerico R, de Wit K, Barco S, Bonorino J, et al. Evidence-based risk stratification of patients with acute pulmonary embolism: communication from the ISTH SSC Subcommittee on Predictive and Diagnostic Variables in Thrombotic Disease J Thromb Haemost, 2026.PMID 41354154
5. [6]Assmann G, Klemm PCM, Hedrich C, Girschick H, et al. [Diagnosis and treatment of chronic nonbacterial osteitis (CNO) and SAPHO syndrome : Implications of the current consensus recommendations of an international commission of experts for German rheumatology] Z Rheumatol, 2026.PMID 41313366
6. [7]Crotti C, Ughi N, Scirè CA, Luppi F, et al. The Italian Society for Rheumatology guidelines for the treatment of patients with rheumatoid arthritis and interstitial lung disease Reumatismo, 2026.PMID 41247141
7. [8]Alexander T, Roldan E, Del Papa N, Farge D, et al. Autologous haematopoietic stem cell transplantation for rheumatic diseases: best practice recommendations from the EBMT Practice Harmonization and Guidelines Committee Bone Marrow Transplant, 2025.PMID 40830237
8. [9]Xanthouli P, Schatz A, Bergner R, Triantafyllias K [Management of pulmonary and cardiovascular comorbidities in rheumatological diseases] Z Rheumatol, 2025.PMID 40736566
9. [10]Liu Y, Ma L, Zhang Z, Zhao Y, et al. 2025 Chinese guideline for the imaging diagnosis and evaluation of Takayasu arteritis Adv Rheumatol, 2025.PMID 40462247
10. [11]Rüegg L, Pluma A, Hamroun S, Cecchi I, et al. EULAR recommendations for use of antirheumatic drugs in reproduction, pregnancy, and lactation: 2024 update Ann Rheum Dis, 2025.PMID 40287311
11. [12]Abu-Zaid MH, Migowa AN, Kassa HL, Messadi W, et al. African guidelines for diagnosis and management of polyarticular juvenile idiopathic arthritis: PAFLAR initiative Pediatr Rheumatol Online J, 2025.PMID 40089767