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

Calculator

When to use

Constitutional symptoms

Absence of the following symptoms

Mild or intermittent fever 37.5-38.5 degrees C)/night sweats and/or involuntary weight loss of 5-10% of body weight

Severe fever > 38.5 degrees C)/night sweats and/or involuntary weight loss of > 10% of body weight

Lymphadenopathy

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

Glandular involvement

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)

Articular involvement

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

Cutaneous involvement

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

Pulmonary involvement

Absence of currently active pulmonary involvement

Persistent cough due to bronchial involvement with no radiographic abnormalities on radiography or radiological; or high-resolution computed tomography evidence of interstitial lung disease with no breathlessness and normal lung function test

Moderately active pulmonary involvement, such as interstitial lung disease shown by high-resolution computed tomography with shortness of breath on exercise New York Heart Association II) or abnormal lung function tests: 70% > diffusion capacity of the lung for carbon monoxide ≥ 40% or 80% > forced vital capacity ≥ 60%

Highly active pulmonary involvement, such as interstitial lung disease shown by high-resolution computed tomography with shortness of breath at rest New York Heart Association III/IV) or with abnormal lung function tests: diffusion capacity of the lung for carbon monoxide < 40% or forced vital capacity < 60%

Renal involvement

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 glomerular filtration rate ≥ 60 mL/min)

Moderately active renal involvement, such as tubular acidosis with renal failure glomerular filtration rate < 60 mL/min) or glomerular involvement with proteinuria 1-1.5 g/day) and without hematuria or renal failure glomerular filtration rate ≥ 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 glomerular filtration rate < 60 mL/min), or histological evidence of proliferative glomerulonephritis or cryoglobulinemia-related renal involvement

Muscular involvement

Absence of currently active muscular involvement

Mild active myositis shown by abnormal electromyography, magnetic resonance imaging or biopsy with no weakness and creatine kinase upper limit of normal ≤ creatine kinase <= 2 times the upper limit of normal)

Moderately active myositis proven by abnormal electromyography, magnetic resonance imaging or biopsy with weakness maximal deficit of 4/5), or elevated creatine kinase 2 times the upper limit of normal < creatine kinase ≤ 4 times the upper limit of normal)

Highly active myositis shown by abnormal electromyography, magnetic resonance imaging or biopsy with weakness deficit ≤ 3/5) or elevated creatine kinase > 4 times the upper limit of normal)

Peripheral nervous system involvement

Absence of currently active peripheral nervous system involvement

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

Moderately active peripheral nervous system 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 peripheral nervous system 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

Central nervous system involvement

Absence of currently active central nervous system involvement

Moderately active central nervous system 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 central nervous system 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

Hematological findings

Absence of autoimmune cytopenia

Cytopenia of autoimmune origin with neutropenia 1000 < neutrophils < 1500/mm**3), and/or anemia 10 < hemoglobin < 12 g/dL), and/or thrombocytopenia 100,000 < platelets < 150,000/mm**3); or lymphopenia 500 < lymphocytes < 1000/mm**3)

Cytopenia of autoimmune origin with neutropenia 500 ≤ neutrophils <= 1000/mm**3), and/or anemia 8 <= hemoglobin <= 10 g/dL), and/or thrombocytopenia 50,000 <= platelets <= 100,000/mm**3); or lymphopenia <= 500/mm**3)

Cytopenia of autoimmune origin with neutropenia neutrophils < 500/mm**3), and/or or anemia haemoglobin < 8 g/dL) and/or thrombocytopenia platelets < 50,000/mm**3)

Biological findings

Absence of any of the following biological features

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

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

Calculation

Please enter all the required inputs.

The EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI score) is a clinical tool specifically designed for patients with Sjögren's Syndrome, a chronic autoimmune disease characterized by dryness of the eyes and mouth. The ESSDAI score is used to assess disease activity and monitor progression over time. It evaluates 12 different domains including constitutional, lymphadenopathy, glandular, articular, cutaneous, pulmonary, renal, muscular, peripheral nervous system, central nervous system, hematological, and biological. Each domain is scored based on the level of activity (low, moderate, high), and the total score provides an overall measure of disease activity. This calculator is not applicable to patients without a confirmed diagnosis of Sjögren's Syndrome.

Reference

Raphaèle Seror, Simon J Bowman, Pilar Brito-Zeron et al. EULAR Sjögren's syndrome disease activity index (ESSDAI): a user guide. RMD Open. 2015 Feb 20;1(1):e000022.

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