Sjogren syndrome
Sjogren syndrome is a systemic autoimmune exocrinopathy in which lymphocytic infiltration of the lacrimal and salivary glands causes keratoconjunctivitis sicca and xerostomia, occurring alone (primary) or alongside another rheumatologic disease such as rheumatoid arthritis or lupus (secondary). Diagnosis combines the anti-Ro (SSA) and anti-La (SSB) antibody panel, objective dryness testing, and labial salivary gland biopsy under the 2016 ACR/EULAR classification criteria. Management centers on sicca-directed therapy, pilocarpine or cevimeline for xerostomia and topical cyclosporine for keratoconjunctivitis, with hydroxychloroquine or immunosuppression for extraglandular disease.
- Topic reviewThe full article, organized the way the exam asks it.
- ABIM-format MCQsVignettes with an explanation for every wrong answer.
- Key factsThe numbers and thresholds worth memorizing, up front.
- AI tutorMedBrain answers the question you're stuck on.
What this article covers
- Overview
Definition: Sjogren syndrome is a systemic autoimmune exocrinopathy in which lymphocytic infiltration of the lacrimal and salivary glands impairs secretion, producing keratoconjunctivitis sicca (dry eyes) and xerostomia (dry mouth).
- Epidemiology
Female predominance: female-to-male ratio of 6 to 9:1.
- Risk factors
Female sex: the dominant demographic risk factor, reflected in the 6 to 9:1 ratio.
- Pathophysiology
Epithelial activation initiates disease: an unknown stimulus activates mucosal epithelial cells, triggering sustained activation of both innate and adaptive immunity.
- Clinical features
Ocular sicca (keratoconjunctivitis sicca): a gritty or foreign-body sensation, itchiness, and redness from insufficient tear film, which risks corneal damage if unmanaged.
- Differential diagnosis
Rheumatoid arthritis: frequently produces secondary Sjogren syndrome, but its own arthritis is symmetric, erosive, small-joint disease with high-titer rheumatoid factor, unlike the nonerosive (or…
- Diagnosis
The diagnosis is established by the 2016 American College of Rheumatology (ACR) and European League Against Rheumatism (EULAR) classification criteria for primary Sjogren syndrome: a weighted…
- Management
First, start nonpharmacologic sicca measures in every patient: hydration, limiting alcohol and caffeinated beverages, smoking cessation, gustatory and mechanical salivary stimulants such as xylitol…
- Complications
Non-Hodgkin lymphoma: an up to 44-fold risk of MALT lymphoma; about 5% of patients develop lymphoma, usually within the first decade, with higher risk when lymphadenopathy, recurrent parotid…
- Prognosis
Favorable overall for primary disease: mortality is not different from the general population.
- Prevention
Hydroxychloroquine retinal screening: a baseline dilated retinal exam within the first year, then annual screening beginning at year 5.
Read the whole article
Subscribing to Internal Medicine opens every article, the full question bank and MedBrain, on the web, iPhone and Android.
Related articles
- Rheumatoid arthritis
Rheumatoid arthritis is a chronic systemic autoimmune disease causing symmetric small-joint polyarthritis of the hands and feet that spares the distal interphalangeal joints, with morning stiffness over an hour. Diagnosis combines the ACR/EULAR classification criteria with rheumatoid factor, anti-CCP antibody, and radiographs. Treat-to-target management anchors on methotrexate, escalating to a biologic or targeted synthetic disease-modifying antirheumatic drug when remission is not reached.
- Systemic lupus erythematosus
Systemic lupus erythematosus is a chronic multisystem autoimmune disease in which antinuclear and other autoantibodies drive immune-complex deposition and organ injury across skin, joints, kidneys, blood, serosa, and the nervous system. Diagnosis rests on a positive antinuclear antibody plus weighted 2019 EULAR/ACR criteria totaling 10 or more points. Hydroxychloroquine is lifelong first-line therapy for nearly every patient, with glucocorticoids and steroid-sparing immunosuppression added for moderate to severe or organ-threatening disease.
- Mixed connective tissue disease
Mixed connective tissue disease is an overlap syndrome defined by a high-titer anti-U1-ribonucleoprotein antibody together with clinical features of systemic lupus erythematosus, systemic sclerosis, and inflammatory myositis, with Raynaud phenomenon and puffy fingers as near-universal early findings. Treatment is organ-directed, built on hydroxychloroquine and glucocorticoids with methotrexate, azathioprine, or mycophenolate mofetil escalation guided by the dominant manifestation.
- Raynaud phenomenon
Raynaud phenomenon is episodic digital vasospasm with triphasic pallor, cyanosis, and reactive hyperemia, split into a benign primary form and a secondary form driven by an underlying connective tissue disease. Diagnosis is clinical, with nailfold capillaroscopy and an autoantibody panel screening for systemic sclerosis, and treatment starts with cold avoidance before a dihydropyridine calcium channel blocker such as nifedipine.
- Non-Hodgkin lymphoma
Non-Hodgkin lymphoma is a heterogeneous group of B-cell (about 85%) and T-cell lymphoid malignancies that spread noncontiguously and often involve extranodal sites, ranging from indolent follicular lymphoma to aggressive diffuse large B-cell lymphoma to highly aggressive Burkitt lymphoma. Diagnosis requires excisional or core biopsy with flow cytometry and cytogenetics, Ann Arbor staging with PET-CT, and treatment is subtype-specific, from observation to R-CHOP to intensive Burkitt regimens with aggressive tumor lysis and CNS prophylaxis.