Sjögren Syndrome
Rheumatology · Sjögren Syndrome 🎯 Practice these cards
Sjögren syndrome has a female-to-male ratio of ____ and peak onset in the ____ decades; the secondary form occurs in ____% of patients with predisposing rheumatic conditions such as RA and SLE.
Sjögren syndrome: female-to-male ratio 6-9:1; peak onset 5th-6th decades (pediatric variant exists). Primary form prevalence 0.5-5 per 1000; secondary Sjögren (most often with RA or SLE) found in 10-30% of predisposing rheumatic disease populations.
A positive Schirmer test is <____ mm of wetting in 5 minutes, and a positive sialometry is unstimulated saliva flow <____ mL/min.
Schirmer test: <5 mm wetting in 5 minutes = ocular dryness. Unstimulated whole saliva flow <0.1 mL/min = oral dryness. Ocular staining score >=5 (one eye) documents corneal damage.
In the 2016 ACR/EULAR criteria, anti-Ro/SSA is worth ____ points, a positive lip biopsy (focus score >=1) is worth ____ points, and classification requires a total >= ____.
Primary Sjögren classification (2016 ACR/EULAR): subjective sicca + score >=4. Weights: labial salivary gland biopsy focus score >=1 = 2; anti-Ro/SSA = 3 (highest); ocular staining score >=5 = 1; Schirmer <=5 mm/5 min = 1; unstimulated saliva <0.1 mL/min = 1. Exclusions: head/neck radiation, active hepatitis C, HIV/AIDS, sarcoidosis, amyloidosis, IgG4-related disease, GVHD.
A positive lip biopsy shows foci of >____ lymphocytes surrounding secretory ducts; focus score >=1 means at least one focus per ____ mm2 of gland tissue.
Labial minor salivary gland biopsy: focal lymphocytic sialadenitis — aggregates of >50 cells (CD4 T cells, B cells, plasma cells) around secretory ducts. Focus score >=1 means at least one such focus per 4 mm2 of gland tissue.
The only autoantibody weighted in the 2016 ACR/EULAR criteria is ____ (____ points); ____ is characteristic of Sjögren syndrome but is not included in the criteria.
Anti-Ro/SSA is specific in PRIMARY Sjögren (less specific in secondary disease, where SLE also produces it) and is the only antibody weighted in the 2016 criteria (3 points). Anti-La/SSB is characteristic of Sjögren syndrome but is NOT included in the classification criteria. ANA + RF co-expression at high titer is more suggestive than either alone.
Sjögren syndrome carries up to a ____-fold increased risk of MALT lymphoma; about ____% of patients develop lymphoma, usually within the first ____ after diagnosis. A previously elevated RF that ____ is a classic serologic red flag.
Up to 44-fold increased risk of MALT lymphoma vs the general population; ~5% of patients develop lymphoma, usually within the first decade after diagnosis. Risk flags: persistent/recurrent parotid swelling, lymphadenopathy, palpable purpura, monoclonal gammopathy, low C4, cryoglobulins, and a falling or disappearing previously elevated rheumatoid factor.
The muscarinic agonists used for moderate-severe oral dryness are ____ and ____; they are contraindicated in ____ glaucoma.
Oral dryness (moderate-severe): muscarinic agonists pilocarpine (5 mg TID) or cevimeline (30 mg TID) — contraindicated in narrow-angle glaucoma. Eyes: artificial tears/gels/ointments first, then topical cyclosporine or a small-molecule integrin antagonist (lifitegrast); lacrimal punctal plugging is widely performed but unproven in trials. Meticulous dental care at all severities.
The most common extraglandular manifestation is ____ (____%), followed by arthralgia/arthritis (____%); the typical kidney lesion is ____ rather than glomerulonephritis.
Extraglandular Sjögren frequencies: fatigue 70%; arthralgia/arthritis 36% (nonerosive arthritis without RA is uncommon); cryoglobulinemia 16%; cutaneous vasculitis 10-16%; thyroid disease 10-15%; interstitial pneumonitis 5-9%; kidney (tubulointerstitial nephritis/type 1 RTA > glomerulonephritis) 5-10%; neurologic (peripheral > central; axonal polyneuropathy typical) 8-27%; systemic vasculitis 7%; Raynaud 4-12%.
HIV-associated sicca shows ____+ T-cell gland infiltrates and lacks anti-Ro/La, whereas Sjögren's disease shows ____+ infiltrates with anti-Ro/La positivity.
HIV-associated sicca syndrome: predominantly young males, CD8+ T-cell salivary infiltrates, HLA-DR5 association, ABSENT anti-Ro/La. Sjögren's disease: middle-aged women, CD4+ T-cell infiltrates, HLA-DR3/DRw52, anti-Ro/La present. Checkpoint inhibitors (anti-PD-1/PD-L1) can also trigger Sjögren's disease.
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