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Sjögren disease

Sjögren disease is an autoimmune disorder affecting exocrine glands (tear and salivary glands) with female predominance (9:1 ratio), typically presenting in the 4th-5th decades with dry eyes and mouth, parotid enlargement, and potential systemic complications including pulmonary, spinal cord, and hematologic involvement.

Key Images

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Look For First
  • Salt-and-pepper or honeycomb appearance of parotid glands on MRI
  • Bilateral parotid gland enlargement with heterogeneous signal and fat infiltration
  • Punctate calcifications within bilateral parotid glands on CT
  • T2 hyperintensity in dorsal columns on spinal cord MRI in patients with sensory neuronopathy
Key Image Findings
  • On ultrasound, early-stage salivary gland involvement may appear normal or enlarged with hyperechoic signal, while late-stage disease characteristically shows multicystic or reticular patterns within atrophic glands.
  • CT imaging reveals typical features including heterogeneous parotid gland parenchyma, abnormal diffuse fat tissue deposition, and bilateral parotid punctate calcifications.
  • MRI of the parotid glands demonstrates salt-and-pepper or honeycomb appearance reflecting glandular heterogeneity and fatty infiltration, with associated changes in lacrimal gland size and accelerated fat deposition.
  • In extraglandular involvement with sensory neuronopathy, MRI spinal cord imaging shows T2 hyperintensity affecting the dorsal columns, which may be longitudinally extensive.
  • Myelitis associated with Sjögren disease presents with transverse myelitis features on MRI, potentially longitudinally extensive and may show neuroradiological features of neuromyelitis optica spectrum disorder (NMOSD).
  • Pulmonary involvement is common (45-75% of patients) with non-specific interstitial pneumonitis (NSIP) being the most frequent pattern, followed by respiratory bronchiolitis, usual interstitial pneumonitis, and lymphocytic interstitial pneumonia.
  • Focal lymphoid hyperplasia and small airways disease may be visible on chest imaging as additional thoracic manifestations.
  • B-cell lymphoma development (typically MALT lymphoma, marginal zone, or diffuse large B-cell type) represents a significant complication requiring surveillance.
Differential Diagnosis
  • HIV-associated benign lymphoepithelial cysts (BLEC) of the parotid gland: distinguished by serology, clinical context, and cystic appearance rather than parenchymal infiltration
  • Other connective tissue diseases (SLE, scleroderma, rheumatoid arthritis): require clinical and serologic correlation; Sjögren disease has characteristic anti-Ro/SSA and anti-La/SSB antibodies
  • Mikulicz syndrome (Sjögren type 1): represents a specific presentation of Sjögren disease with symmetric parotid/lacrimal gland enlargement
  • Sialadenitis from infection (EBV, hepatitis C, HTLV): distinguished by acute clinical presentation and infectious serologies
  • Lymphoma: must be distinguished from the underlying Sjögren disease; imaging and biopsy may be needed to confirm transformation
  • Sarcoidosis: may present with parotid enlargement but lacks characteristic glandular fat infiltration and reticular patterns of Sjögren disease
Discussion

Sjögren disease is the second most common autoimmune disorder after rheumatoid arthritis, with strong female predominance (9:1), making it important to screen women in the 4th-5th decades presenting with dry eye and dry mouth symptoms.

The disease involves B-lymphocyte hyperactivity and autoantibody production (anti-Ro/SSA, anti-Ro52, anti-Ro60, anti-La/SSB), distinguishing it from other connective tissue diseases and making serologic testing essential for diagnosis.

Extraglandular manifestations occur in a significant portion of patients, including pulmonary disease (NSIP most common at 45%), myelitis with dorsal column involvement, and increased risk of B-cell lymphoma (4-8% of patients), requiring multisystem surveillance.

Pulmonary involvement is the most common extraglandular manifestation, with NSIP occurring in 45% of patients, followed by respiratory bronchiolitis (25%), usual interstitial pneumonitis (16%), and lymphocytic interstitial pneumonia (15%).

Spinal cord involvement presenting as sensory neuronopathy or myelitis may show longitudinally extensive T2 hyperintensity in the dorsal columns and can be associated with neuromyelitis optica spectrum disorder, requiring careful neuroimaging follow-up.

Current treatment is not disease-modifying for early glandular disease; active monitoring for extraglandular involvement is critical as the prognosis depends on controlling disease activity and preventing serious systemic complications.

Reporting Pearls

Describe parotid gland involvement as showing characteristic 'salt-and-pepper' or 'honeycomb' appearance on MRI reflecting interspersed fatty infiltration and fibrosis, and note bilateral punctate calcifications on CT as a specific finding supporting diagnosis; always screen for systemic involvement including pulmonary interstitial changes, dorsal column T2 hyperintensity, and lymphadenopathy indicating possible lymphoma development.

Pitfalls
  • Mistaking early-stage Sjögren disease (which may show normal or only mildly enlarged parotid glands) as normal, when subtle hyperechoic signal on ultrasound or early salt-and-pepper appearance on MRI may be the only clue.
  • Overlooking systemic manifestations, particularly pulmonary interstitial pneumonitis (present in up to 75% of patients) and spinal cord involvement, which require separate dedicated imaging and have significant prognostic implications.
  • Confusing Sjögren disease with Mikulicz syndrome (Sjögren type 1), which is actually a specific presentation of Sjögren disease with prominent symmetric parotid and lacrimal gland enlargement rather than a separate entity.
  • Failing to distinguish between inflammatory glandular changes and lymphoma development; new heterogeneous masses, rapid enlargement, or loss of normal glandular architecture should raise concern for transformation and warrant biopsy.
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