The EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI): domain and item definitions and scores
| Domain | Activity level | Description |
|---|---|---|
| Constitutional Exclusion of fever of infectious origin and voluntary weight loss | No=0 | Absence of the following symptoms |
| Low=3 | Mild or intermittent fever (37.5°–38.5°C)/night sweats and/or involuntary weight loss of 5–10% of body weight | |
| Moderate=6 | Severe fever (>38.5°C) / night sweats and/or involuntary weight loss of >10% of body weight | |
| Lymphadenopathy Exclusion of infection | No=0 | Absence of the following features |
| Low=4 | Lymphadenopathy ≥1 cm in any nodal region or ≥2 cm in inguinal region | |
| Moderate=8 | Lymphadenopathy ≥2 cm in any nodal region or ≥3 cm in inguinal region, and/or splenomegaly (clinically palpable or assessed by imaging) | |
| High=12 | Current malignant B-cell proliferative disorder* | |
| Glandular Exclusion of stone or infection | No=0 | Absence of glandular swelling |
| Low=2 | Small glandular swelling with enlarged parotid (≤3 cm), or limited submandibular or lachrymal swelling | |
| Moderate=4 | Major glandular swelling with enlarged parotid (>3 cm), or important submandibular or lachrymal swelling | |
| Articular Exclusion of osteoarthritis | No=0 | Absence of currently active articular involvement |
| Low=2 | Arthralgias in hands, wrists, ankles and feet accompanied by morning stiffness (>30 min) | |
| Moderate=4 | 1–5 (of 28 total count) synovitis | |
| High=6 | ≥6 (of 28 total count) synovitis | |
| Cutaneous Rate as ‘No activity’ stable long-lasting features related to damage | No=0 | Absence of currently active cutaneous involvement |
| Low=3 | Erythema multiforma | |
| Moderate=6 | Limited cutaneous vasculitis, including urticarial vasculitis, or purpura limited to feet and ankle, or subacute cutaneous lupus | |
| High=9 | Diffuse cutaneous vasculitis, including urticarial vasculitis, or diffuse purpura, or ulcers related to vasculitis | |
| Pulmonary Rate as ‘No activity’ stable long-lasting features related to damage, or respiratory involvement not related to the disease (tobacco use, etc.) | No=0 | Absence of currently active pulmonary involvement |
| Low=5 | Persistent cough or bronchial involvement with no radiographic abnormalities on radiography Or radiological or HRCT evidence of interstitial lung disease with: No breathlessness and normal lung function test. | |
| Moderate=10 | Moderately active pulmonary involvement, such as interstitial lung disease shown by HRCT with shortness of breath on exercise (NHYA II) or abnormal lung function tests restricted to: 70%>DLCO ≥ 40% or 80%>FVC≥60% | |
| High=15 | Highly active pulmonary involvement, such as interstitial lung disease shown by HRCT with shortness of breath at rest (NHYA III, IV) or with abnormal lung function tests: DLCO < 40% or FVC<60% | |
| Renal Rate as ‘No activity’ stable long-lasting features related to damage, and renal involvement not related to the disease. If biopsy has been performed, please rate activity based on histological features first | No=0 | Absence of currently active renal involvement with proteinuria <0.5 g/day, no haematuria, no leucocyturia, no acidosis, or long-lasting stable proteinuria due to damage |
| Low=5 | Evidence of mild active renal involvement, limited to tubular acidosis without renal failure or glomerular involvement with proteinuria (between 0.5 and 1 g/day) and without haematuria or renal failure (GFR ≥60 mL/min) | |
| Moderate=10 | Moderately active renal involvement, such as tubular acidosis with renal failure (GFR <60 mL/min) or glomerular involvement with proteinuria between 1 and 1.5 g/day and without haematuria or renal failure (GFR ≥60 mL/min) or histological evidence of extra-membranous glomerulonephritis or important interstitial lymphoid infiltrate | |
| High=15 | Highly active renal involvement, such as glomerular involvement with proteinuria >1.5 g/day or haematuria or renal failure (GFR <60 mL/min), or histological evidence of proliferative glomerulonephritis or cryoglobulinemia related renal involvement | |
| Muscular Exclusion of weakness due to corticosteroids | No=0 | Absence of currently active muscular involvement |
| Low=6 | Mild active myositis shown by abnormal EMG or biopsy with no weakness and creatine kinase (N<CK≤2N) | |
| Moderate=12 | Moderately active myositis proven by abnormal EMG or biopsy with weakness (maximal deficit of 4/5), or elevated creatine kinase (2N <CK ≤4N), | |
| High=18 | Highly active myositis shown by abnormal EMG or biopsy with weakness (deficit ≤3/5) or elevated creatine kinase (>4N) | |
| PNS Rate as ‘No activity’ stable long-lasting features related to damage or PNS involvement not related to the disease | No=0 | Absence of currently active PNS involvement |
| Low=5 | Mild active peripheral nervous system involvement, such as pure sensory axonal polyneuropathy shown by NCS or trigeminal (V) neuralgia | |
| Moderate=10 | Moderately active peripheral nervous system involvement shown by NCS, such as axonal sensory-motor neuropathy with maximal motor deficit of 4/5, pure sensory neuropathy with presence of cryoglobulinemic vasculitis, ganglionopathy with symptoms restricted to mild/moderate ataxia, inflammatory demyelinating polyneuropathy (CIDP) with mild functional impairment (maximal motor deficit of 4/5or mild ataxia), Or cranial nerve involvement of peripheral origin (except trigeminal (V) neuralgia) | |
| High=15 | Highly active PNS involvement shown by NCS, such as axonal sensory-motor neuropathy with motor deficit ≤3/5, peripheral nerve involvement due to vasculitis (mononeuritis multiplex, etc), severe ataxia due to ganglionopathy, inflammatory demyelinating polyneuropathy (CIDP) with severe functional impairment: motor deficit ≤3/5 or severe ataxia | |
| CNS Rate as ‘No activity’ stable long-lasting features related to damage or CNS involvement not related to the disease | No=0 | Absence of currently active CNS involvement |
| Moderate=10 | 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 | |
| High=15 | Highly active CNS features, such as cerebral vasculitis with cerebrovascular accident or transient ischaemic attack, seizures, transverse myelitis, lymphocytic meningitis, multiple sclerosis-like syndrome with motor deficit. | |
| Haematological For anaemia, neutropenia, and thrombopenia, only autoimmune cytopenia must be considered Exclusion of vitamin or iron deficiency, drug-induced cytopenia | No=0 | Absence of autoimmune cytopenia |
| Low=2 | Cytopenia of autoimmune origin with neutropenia (1000<neutrophils<1500/mm3), and/or anaemia (10<haemoglobin<12 g/dL), and/or thrombocytopenia (100 000<platelets<150 000/mm3) Or lymphopenia (500<lymphocytes<1000/mm3) | |
| Moderate=4 | Cytopenia of autoimmune origin with neutropenia (500≤neutrophils≤1000/mm3), and/or anaemia (8≤haemoglobin≤10 g/dL), and/or thrombocytopenia (50 000≤platelets≤100 000/mm3) Or lymphopenia (≤500/mm3) | |
| High=6 | Cytopenia of autoimmune origin with neutropenia (neutrophils <500/mm3), and/or or anaemia (haemoglobin <8 g/dL) and/or thrombocytopenia (platelets <50 000/mm3) | |
| Biological | No=0 | Absence of any of the following biological features |
| Low=1 | Clonal component and/or hypocomplementemia (low C4 or C3 or CH50) and/or hypergammaglobulinemia or high IgG level between 16 and 20 g/L | |
| Moderate=2 | 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) |
*Defined as indolent not treated lymphoma or currently treated lymphoma or myeloma (or treatment ended from less than 6 months). Do not rate past treated lymphoma or myeloma in complete remission.
CIDP, chronic inflammatory demyelinating polyneuropathy; CK, creatine kinase; CNS, central nervous system; DLCO, diffusing CO capacity; EMG, electromyogram; FVC, forced vital capacity; GFR, glomerular filtration rate; Hb, haemoglobin; HRCT, high-resolution CT; IgG, immunoglobulin G; NCS, nerve conduction studies; NHYA, New York Heart Association Classification; Plt, platelet; PNS, peripheral nervous system.