Rheumatology · Sjögren Syndrome · study mode — pick an answer, then reveal.
Question · easy
A 54-year-old woman reports 2 years of gritty eyes and dry mouth requiring water to swallow dry food. Schirmer test is 3 mm/5 min bilaterally. Anti-Ro/SSA antibodies are positive; anti-La/SSB, rheumatoid factor, and ANA are negative. She has no history of head/neck radiation, hepatitis C, HIV, sarcoidosis, or IgG4-related disease. Using the 2016 ACR/EULAR classification criteria for primary Sjögren syndrome, which statement is correct?
The 2016 ACR/EULAR criteria require subjective sicca plus a weighted score >=4 after exclusions are ruled out. Anti-Ro/SSA carries 3 points (the single highest weight) and a Schirmer test <=5 mm/5 min carries 1 point: 3 + 1 = 4, so she is classified. Anti-La/SSB is characteristic but is NOT part of the criteria. Biopsy (2 points) is an alternative route, not a requirement. No single additional test is mandatory once the threshold is reached.
MKSAP 19 B.6 Ch.7; Harrison's 22e Ch.373
Question · easy
A 48-year-old woman with established primary Sjögren syndrome asks what extraglandular problems are most likely to affect her. Which of the following is the most common extraglandular manifestation of Sjögren syndrome?
Fatigue affects roughly 70% of patients and is the most common extraglandular manifestation; arthralgia/arthritis follows at about 36%. Interstitial pneumonitis (5-9%), peripheral neuropathy including mononeuritis multiplex (part of the 8-27% neurologic spectrum), and glomerulonephritis (within the 5-10% renal spectrum, where tubulointerstitial nephritis predominates) are all substantially less common.
MKSAP 19 B.6 Ch.7
Question · medium
A 61-year-old woman with a 9-year history of primary Sjögren syndrome is reviewed in clinic. Which constellation of findings should most urgently raise suspicion that she is developing lymphoma?
The classic lymphoma-risk signature in Sjögren syndrome combines clinical (persistent/recurrent parotid swelling, lymphadenopathy, palpable purpura), serologic (low C4, monoclonal gammopathy, cryoglobulins, and a falling or disappearing previously elevated RF), and histopathologic (germinal-center infiltrates) features. MALT lymphoma risk is elevated up to 44-fold; about 5% of patients develop lymphoma, mostly in the first decade. Stable sicca, a positive focus score, neuropathy, or stable polyclonal hypergammaglobulinemia do not carry the same alarm.
MKSAP 19 B.6 Ch.7; Harrison's 22e Ch.373
Question · medium
A 57-year-old man with primary Sjögren syndrome has moderate-to-severe oral dryness despite sugar-free gum and candies. His ophthalmologist has just diagnosed narrow-angle glaucoma. Which is the most appropriate next step for his oral sicca?
The muscarinic agonists pilocarpine and cevimeline are the pharmacologic mainstay for moderate-to-severe oral dryness but are contraindicated in narrow-angle glaucoma. With both excluded, management falls back on meticulous dental care, nonpharmacologic salivary stimulation, and (limited-benefit) artificial saliva. Punctal plugging addresses ocular (not oral) dryness and, in any case, has not shown efficacy in trials.
MKSAP 19 B.6 Ch.7; Harrison's 22e Ch.373
Question · hard
A 66-year-old man presents with 18 months of progressive bilateral parotid and submandibular gland enlargement and mild dry eyes. Anti-Ro/SSA and anti-La/SSB are negative; ANA is weakly positive. Schirmer test is 6 mm/5 min bilaterally. He has no fever, weight loss, or night sweats. Which is the most important condition to exclude before diagnosing Sjögren syndrome?
In an older man with bilateral major-gland enlargement, negative Ro/La, and only borderline objective dryness, IgG4-related disease is the key mimic and is an explicit exclusion in the 2016 ACR/EULAR criteria (along with head/neck radiation, hepatitis C, HIV/AIDS, sarcoidosis, amyloidosis, and graft-versus-host disease); serum IgG4 and histology (storiform fibrosis, IgG4+ plasma cells rather than lymphoepithelial sialadenitis) discriminate. RA and fibromyalgia do not cause gland enlargement. Checkpoint inhibitor-triggered Sjögren's is real but requires an exposure history (anti-PD-1/PD-L1 therapy), which is not present here.