EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI score)

Absence of the following features

Lymphadenopathy ≥ 1 cm in any nodal region or ≥ 2 cm in the inguinal region

Lymphadenopathy ≥ 2 cm in any nodal region or ≥ 3 cm in the inguinal region, and/or palpable or imaging-confirmed splenomegaly

Current malignant B-cell proliferative disorder

Absence of glandular swelling

Small glandular swelling with enlarged parotid (≤ 3 cm), or limited submandibular (≤ 2 cm) or lachrymal swelling (≤ 1 cm)

Major glandular swelling with enlarged parotid (> 3 cm), or important submandibular (> 2 cm) or lachrymal swelling (> 1 cm)

Absence of currently active articular involvement

Arthralgias in hands, wrists, ankles, and feet accompanied by morning stiffness (> 30 min)

1-5 (of 28 total count) synovitis

≥ 6 (of 28 total count) synovitis

Absence of currently active cutaneous involvement

Erythema multiforme

Limited cutaneous vasculitis, including urticarial vasculitis, or purpura limited to feet and ankle, or subacute cutaneous lupus

Diffuse cutaneous vasculitis, including urticarial vasculitis, or diffuse purpura, or ulcers related to vasculitis

Absence of currently active pulmonary involvement

Persistent cough due to bronchial involvement with no radiographic abnormalities on radiography or radiological; or HRCT evidence of ILD with no breathlessness and normal lung function test

Moderately active pulmonary involvement, such as ILD shown by HRCT with shortness of breath on exercise (NYHA II) or abnormal lung function tests: 70% > DLCO ≥ 40% or 80% > FVC ≥ 60%

Highly active pulmonary involvement, such as ILD shown by HRCT with shortness of breath at rest (NYHA III/IV) or with abnormal lung function tests: DLCO < 40% or FVC < 60%

Absence of currently active renal involvement with proteinuria < 0.5 g/day, no hematuria, no leukocyturia, no acidosis or long-lasting stable proteinuria due to damage

Evidence of mild active renal involvement, limited to tubular acidosis without renal failure or glomerular involvement with proteinuria (0.5-1 g/day) and without hematuria or renal failure (GFR ≥ 60 mL/min)

Moderately active renal involvement, such as tubular acidosis with renal failure (GFR < 60 mL/min) or glomerular involvement with proteinuria (1-1.5 g/day) and without hematuria or renal failure (GFR ≥ 60 mL/min) or histological evidence of extra-membranous glomerulonephritis or important interstitial lymphoid infiltrate

Highly active renal involvement, such as glomerular involvement with proteinuria (> 1.5 g/day), or hematuria or renal failure (GFR < 60 mL/min), or histological evidence of proliferative glomerulonephritis or cryoglobulinemia-related renal involvement

Absence of currently active muscular involvement

Mild active myositis shown by abnormal EMG, MRI or biopsy with no weakness and CK (ULN ≤ CK ≤ 2 times the ULN)

Moderately active myositis proven by abnormal EMG, MRI or biopsy with weakness (maximal deficit of 4/5), or elevated CK (2 times the ULN < CK ≤ 4 times the ULN)

Highly active myositis shown by abnormal EMG, MRI or biopsy with weakness (deficit ≤ 3/5) or elevated CK (> 4 times the ULN)

Absence of currently active PNS involvement

Mild active PNS involvement, such as pure sensory axonal polyneuropathy shown by nerve conduction study, or trigeminal neuralgia

Moderately active PNS involvement shown by nerve conduction study, such as axonal sensory-motor neuropathy with a maximal motor deficit of 4/5, pure sensory neuropathy with the presence of cryoglobulinemic vasculitis, ganglionopathy with symptoms restricted to mild/moderate ataxia, inflammatory demyelinating polyneuropathy with mild functional impairment (maximal motor deficit of 4/5 or mild ataxia); or cranial nerve involvement of peripheral origin (except trigeminal neuralgia)

Highly active PNS involvement shown by nerve conduction study, such as axonal sensory-motor neuropathy with motor deficit ≤ 3/5, peripheral nerve involvement due to vasculitis (such as mononeuritis multiplex), severe ataxia due to ganglionopathy, inflammatory demyelinating polyneuropathy with severe functional impairment: motor deficit ≤ 3/5 or severe ataxia

Absence of currently active CNS involvement

Moderately active CNS features, such as cranial nerve involvement of central origin, optic neuritis, or multiple sclerosis-like syndrome with symptoms restricted to pure sensory impairment or proven cognitive impairment

Highly active CNS features, such as cerebral vasculitis with a cerebrovascular accident or transient ischaemic attack, seizures, transverse myelitis, lymphocytic meningitis, multiple sclerosis-like syndrome with motor deficit

Absence of autoimmune cytopenia

Cytopenia of autoimmune origin with neutropenia (1000 < neutrophils < 1500/mm³), and/or anemia (10 < hemoglobin < 12 g/dL), and/or thrombocytopenia (100,000 < platelets < 150,000/mm³); or lymphopenia (500 < lymphocytes < 1000/mm³)

Cytopenia of autoimmune origin with neutropenia (500 ≤ neutrophils ≤ 1000/mm³), and/or anemia (8 ≤ hemoglobin ≤ 10 g/dL), and/or thrombocytopenia (50,000 ≤ platelets ≤ 100,000/mm³); or lymphopenia (≤ 500/mm³)

Cytopenia of autoimmune origin with neutropenia (neutrophils < 500/mm³), and/or or anemia (haemoglobin < 8 g/dL) and/or thrombocytopenia (platelets < 50,000/mm³)

Absence of any of the following biological features

Clonal component and/or hypocomplementemia (low C4 or C3 or CH50) and/or hypergammaglobulinemia or high IgG level 16-20 g/L

Presence of cryoglobulinemia and/or hypergammaglobulinemia or high IgG level > 20 g/L, and/or recent-onset hypogammaglobulinemia or recent decrease of IgG level (< 5 g/L)