SJ Gren S 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). [9] [4]

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 incorporating anti-Ro/SSA and anti-La/SSB antibodies, ocular staining score, salivary flow rate, and minor salivary gland biopsy — 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. [4] [11]

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