The EULAR Sjögren's syndrome disease activity index (ESSDAI): domain and item definitions and weights
| Domain [weight] | Activity level | Description |
|---|---|---|
| Constitutional [3] | No = 0 | Absence of the following symptoms |
| Exclusion of fever of infectious origin and voluntary weight loss | Low = 1 | Mild or intermittent fever (37.5–38.5°C)/night sweats and/or involuntary weight loss of 5–10% of body weight |
| Moderate = 2 | Severe fever (>38.5°C)/night sweats and/or involuntary weight loss of >10% of body weight | |
| Lymphadenopathy [4] | No = 0 | Absence of the following features |
| Exclusion of infection | Low = 1 | Lymphadenopathy ≥1 cm in any nodal region or ≥2 cm in inguinal region |
| Moderate = 2 | Lymphadenopathy ≥2 cm in any nodal region or ≥3 cm in inguinal region, and/or splenomegaly (clinically palpable or assessed by imaging) | |
| High = 3 | Current malignant B-cell proliferative disorder | |
| Glandular [2] | No = 0 | Absence of glandular swelling |
| Exclusion of stone or infection | Low =1 | Small glandular swelling with enlarged parotid (≤3 cm), or limited submandibular or lachrymal swelling |
| Moderate = 2 | Major glandular swelling with enlarged parotid (>3 cm), or important submandibular or lachrymal swelling | |
| Articular [2] | No = 0 | Absence of currently active articular involvement |
| Exclusion of osteoarthritis | Low = 1 | Arthralgias in hands, wrists, ankles and feet accompanied by morning stiffness (>30 min) |
| Moderate = 2 | 1–5 (of 28 total count) synovitis | |
| High = 3 | ≥6 (of 28 total count) synovitis | |
| Cutaneous [3] | No = 0 | Absence of currently active cutaneous involvement |
| Rate as ‘no activity’ stable long-lasting features related to damage | Low =1 | Erythema multiforma |
| Moderate = 2 | Limited cutaneous vasculitis, including urticarial vasculitis, or purpura limited to feet and ankle, or subacute cutaneous lupus | |
| High = 3 | Diffuse cutaneous vasculitis, including urticarial vasculitis, or diffuse purpura, or ulcers related to vasculitis | |
| Pulmonary [5] | No =0 | Absence of currently active pulmonary involvement |
| Rate as ‘no activity’ stable long-lasting features related to damage, or respiratory involvement not related to the disease (tobacco use, etc) | Low = 1 | 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 = 2 | 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 = 3 | 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 [5] | 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 |
| Rate as ‘no activity’ stable long-lasting features related to damage and renal involvement not related to the disease. | Low = 1 | 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) |
| If biopsy has been performed, please rate activity based on histological features first | ||
| Moderate = 2 | 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 = 3 | 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 cryoglobulinaemia-related renal involvement | |
| Muscular [6] | No = 0 | Absence of currently active muscular involvement |
| Exclusion of weakness due to corticosteroids | Low = 1 | Mild active myositis shown by abnormal EMG or biopsy with no weakness and creatine kinase (N<CK≤2N) |
| Moderate = 2 | Moderately active myositis confirmed by abnormal EMG or biopsy with weakness (maximal deficit of 4/5), or elevated creatine kinase (2N<CK≤4N) | |
| High = 3 | Highly active myositis shown by abnormal EMG or biopsy with weakness (deficit ≤3/5) or elevated creatine kinase (>4N) | |
| PNS [5] | No = 0 | Absence of currently active PNS involvement |
| Rate as ‘no activity’ stable long-lasting features related to damage or PNS involvement not related to the disease | Low = 1 | Mild active peripheral nervous system involvement, such as pure sensory axonal polyneuropathy shown by NCS or trigeminal (V) neuralgia |
| Moderate = 2 | Moderately active peripheral nervous system involvement shown by NCS, such as axonal sensorimotor neuropathy with maximal motor deficit of 4/5, pure sensory neuropathy with presence of cryoglobulinamic vasculitis, ganglionopathy with symptoms restricted to mild/moderate ataxia, inflammatory demyelinating polyneuropathy (CIDP) with mild functional impairment (maximal motor deficit of 4/5 or mild ataxia) | |
| Or cranial nerve involvement of peripheral origin (except trigeminal (V) neralgia) | ||
| High = 3 | Highly active PNS involvement shown by NCS, such as axonal sensorimotor 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 [5] | No = 0 | Absence of currently active CNS involvement |
| Rate as ‘no activity’ stable long-lasting features related to damage or CNS involvement not related to the disease | Low = 1 | 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 confirmed cognitive impairment |
| High = 3 | 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 [2] | No = 0 | Absence of auto-immune cytopenia |
| For anaemia, neutropenia, and thrombopenia, only autoimmune cytopenia must be considered | Low = 1 | Cytopenia of auto-immune origin with neutropenia (1000<neutrophils<1500/mm3), and/or anaemia (10<haemoglobin<12 g/dl), and/or thrombocytopenia (100000<platelets<150000/mm3) |
| Or lymphopenia (500<lymphocytes<1000/mm3) | ||
| Exclusion of vitamin or iron deficiency, drug-induced cytopenia | Moderate = 2 | Cytopenia of auto-immune origin with neutropenia (500≤neutrophils≤1000/mm3), and/or anaemia (8≤haemoglobin≤10 g/dl), and/or thrombocytopenia (50000≤platelets≤100000/mm3) |
| Or lymphopenia (≤500/mm3) | ||
| High = 3 | Cytopenia of auto-immune origin with neutropenia (neutrophils <500/mm3), and/or or anaemia (haemoglobin <8 g/dl) and/or thrombocytopenia (platelets <50000/mm3) | |
| Biological [1] | No = 0 | Absence of any of the following biological features |
| Low = 1 | Clonal component and/or hypocomplementaemia (low C4 or C3 or CH50) and/or hypergammaglobulinaemia or high IgG level between 16 and 20 g/l | |
| Moderate = 2 | Presence of cryoglobulinaemia and/or hypergammaglobulinaemia or high IgG level >20 g/l, and/or recent onset hypogammaglobulinaemia or recent decrease of IgG level (<5 g/l) |
CIDP, chronic inflammatory demyelinating polyneuropathy; CK, creatine kinase; CNS, central nervous system; DLCO, diffusing CO capacity; EMG, electromyogram; EULAR, European League Against Rheumatism; FVC, forced vital capacity; GFR, glomerular filtration rate; Hb, haemoglobin; HRCT, high-resolution computed tomography; IgG, immunoglobulin G; NCS, nerve conduction studies; NHYA, New York Heart Association classification; Plt, platelet; PNS, peripheral nervous system.