Sjögren’s disease-associated ILD

Definitions & Classifications

  • Sjögren’s: Autoimmune destruction of the lacrimal and salivary glands due to an unclear underlying mechanism, but likely due to cell-mediated autoimmunity (based on known HLA associations)

  • Sjögren’s can be primary or secondary

    • Primary Sjogren’s: Does not meet criteria for another SARD

    • Secondary Sjogren’s: Occurring in setting of another SARD (RA, SLE >> SSc, IIM)

  • Prevalence about 1:1000; F:M = 9:1

Clinical Features of Sjögren’s

  • Glandular features (“sicca”)

    • Severe dry eyes, including corneal ulceration

    • Dry mouth, often causing dental caries

  • Non-glandular features include constitutional, dermatologic, articular, pulmonary, renal, vascular, neurologic, gastrointestinal, and hematologic findings

  • Antibodies: Anti-Ro (SSA) and anti-LA (SSB) have distinct test characteristics

    • Ro (SSA): 30-70% of Sjögren’s patients; sometimes present in SLE; poor prognostic factor if present with IIM-ILD. More common in primary than secondary Sjogren’s.

    • La (SSB): 25-40% of Sjögren’s patients; uncommon in SLE. Rarely present if anti-Ro is not.

  • Diagnosis: Usually by 2017 ACR/EULAR Sjögren’s classification criteria; must have sicca symptoms + >4 points

    • 3 points, Salivary gland biopsy with at least one focus of lymphocytic infiltrate

    • 3 points, Anti-Ro/SSA autoantibodies

    • 1 point, Fluorescein/lissamine ocular staining score ≥5 (0–12) in at least one eye

    • 1 point, Schirmer test ≤5 mm/5 min in at least one eye

    • 1 point, Unstimulated whole saliva flow rate ≤0.1 mL/min

  • Mimics (due to ability to cause sicca-like symptoms):

    • Inflammation/malignancy with salivary gland tropism: sarcoidosis, IgG4-RD, GPA, lymphoma

    • Viral infections with salivary gland tropism: mumps, HCV, HIV

    • Medications: anticholinergics, cannabinoids

Pulmonary manifestations of Sjögren’s

  • Airway disorders (xerotrachea, bronchiectasis, bronchiolitis)

  • Interstitial lung disease

  • Drug-induced lung disease (e.g., due to Sjögren’s directed therapy)

  • Pulmonary lymphoproliferative disorders (lymphoma, amyloidosis)

Sjögren’s disease-associated ILD (SjD-ILD)

Diagnosis

  • Imaging Patterns: NSIP >> RB/AMP > UIP, LIP, OP

    • NSIP pattern is most common (~40%); as noted, UIP pattern is uncommon but possible

    • LIP pattern is uncommon but, among SARD, the LIP pattern is most strongly associated with SjD and RA

      • SjD with isolated lung cysts → highly suggestive of LIP

      • SjD with lung cystic changes and associated nodules → consider amyloid or MALT lymphoma

Management

  • Chronic management

    • Screening:

    • Monitoring:

    • Pharmacologic Interventions:

  • Rapidly progressive or exacerbating ILD

    • Approach:

    • Pharmacologic Interventions:

Prognosis

  • Calculators

References