Table 2

Glossary and definitions

1.Nomenclature of therapies
1.1. Topical therapies
1.2. Systemic therapies
1.1. Interventions directly applied to the mucosal surfaces involved
1.2. Drugs administered orally or intravenously for systemic disease
1.1. Saliva substitutes, ocular tears
1.2. Antimalarials, glucocorticoids, immunosuppressive agents, intravenous immunoglobulins, biologics
2.Disease activity terms
2.1. Systemic disease
2.2. Active systemic disease
2.3. Severe systemic disease
2.4. Refractory systemic disease
2.5. Therapeutic response
2.1. Disease involvement that affects or has affected any of the organs/systems included in the clinESSDAI score
2.2. Patients with clinESSDAI score ≥1.
2.3. Patients with ESSDAI score >14, or high activity in any of the ESSDAI domains with a definition of high activity
2.4. Systemic manifestation/s refractory to SOC.
2.5. Decrease of ≥3 points in the global ESSDAI score
2.1. All ESSDAI domains except biological domain
2.2. Systemic activity is classified as low if ESSDAI is 1–4 (if not only due to biological domain), moderate between 5–13 and high ≥14.
2.3. Lymphadenopathy and lymphoma, articular, cutaneous, pulmonary, renal, muscular central and peripheral neurological and haematological domains.
2.4. Due to the diversity of systemic manifestations, SOC (first-line therapeutic approach) has been defined for each systemic manifestation (figure 3)
3.Ocular dryness
3.1. Refractory
3.2. Severe
3.1. Refractory ocular dryness is defined as not improvement after using the best-available SOC and ruling out other SjS-unrelated processes,
3.2. Severity should be defined after a specific ophthalmological evaluation of corneal damage by ocular scores:
3.1. SOC defined as the maximum use of artificial tears and ointments according to the previous recommendation
3.2. Measurement of the OSS and OSDI ocular scores
4.Recommended instruments of measure
4.1. Salivary gland function
4.2. Corneal damage
4.3. Fatigue
4.4. Pain
4.5. Quality of life
4.6. Systemic disease
4.2. OSS, OSDI
4.3. ESSPRI domains, ProFAD
4.4. ESSPRI domains, BPI
5.Potential life-threatening systemic manifestations5.1. Cutaneous domain
5.2. Pulmonary domain
5.3. Renal domain
5.4. Muscular domain
5.5. Peripheral nerve system domain
5.6. CNS domain
5.7. Haematological domain
5.1. Diffuse vasculitis with ulcers
5.2. ILD with NHYA III/IV
5.3. Renal failure; rapidly-progressive glomerulonephritis; hypokalaemic paralysis
5.4. Muscular involvement with severe weakness
5.5. Neuropathy (including ganglionopathy and polyradiculopathies) with severe motor deficit/ataxia; cryoglobulinemic-related multineuritis
5.6. Demyelinating disease with motor deficit; cerebral vasculitis presenting with focal deficit; myelitis; meningoencephalitis
5.7. Severe haemolytic anaemia (<80 g/dL, <50 x109/L); severe autoimmune thrombocytopenia (<50 000/mm3)
  • BPI, brief pain inventory; CHB, congenital heart block; CIDP, chronic inflammatory demyelinating polyradiculopathy; CNS, central nervous system; ESSDAI, EULAR Sjögren's Syndrome Disease Activity Index; ESSPRI, EULAR Sjögren's Syndrome Patient Reported Index; EULAR, European League Against Rheumatism; G-CSF, granulocyte colony-stimulating factor; ILD, interstitial lung disease; KCS, keratoconjunctivitis sicca; LIP, lymphoid interstitial pneumonitis; MMF, mycophenolate mofetil; MP, methylpredisolone; MS, multiple sclerosis; NAC, N-acetylcysteine; NHYA, New York Heart Association; NMOSD, neuromyelitis optica spectrum disorder; NSIP, non-specific interstitial pneumonitis; OP, organising pneumonitis; OSDI, Ocular Surface Disease Index; OSS, Ocular Staining Score; PN, peripheral neuropathy; ROR, retinoic acid-related orphan receptor; RTX, rituximab; SjS, Sjögren syndrome; SOC, standard of care; STAT, signal transducer and activator of transcriptionm; SWSF, stimulated whole salivary flows; TOR, mammalian target of rapamycin; UIP, usual interstitial pneumonitis; UWSF, unstimulated whole salivary flows.