Version May 2024
| Domain | Exclusion(s) and notes | 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 to 38.5°C)/night sweats and/or involuntary weight loss of 5 to 10% of body weight | ||
| Moderate = 6 | Fever (>38.5°C)/night sweats and/or involuntary weight loss of >10% of body weight | ||
| Lymphadenopathy and lymphoma | 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 (≤2 cm) or lacrimal swelling (≤1 cm) | ||
| Moderate = 4 | Major glandular swelling with enlarged parotid (>3 cm), or important submandibular (>2 cm) or lacrimal swelling (>1 cm) | ||
| 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 minutes) | ||
| Moderate = 4 | 1 to 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 multiforme | ||
| Moderate = 6 | Limited cutaneous vasculitis, including urticarial vasculitis, or purpura limited to feet and ankles, 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 due to bronchial involvement with no radiographic abnormalities on radiography or Radiologic 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 (NYHA II) or abnormal lung function tests restricted to DLCO <70% and ≥40% or FVC <80% and ≥60% | ||
| High = 15 | Highly active pulmonary involvement, such as interstitial lung disease shown by HRCT with shortness of breath at rest (NYHA 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 histologic features first. | No = 0 | 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 |
| 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 hematuria or renal failure (GFR ≥60 mL/minute) | ||
| Moderate = 10 | Moderately active renal involvement, such as tubular acidosis with renal failure (GFR <60 mL/minute) or glomerular involvement with proteinuria between 1 and 1.5 g/day and without hematuria or renal failure (GFR ≥60 mL/minute) or histologic evidence of extramembranous glomerulonephritis or important interstitial lymphoid infiltrate | ||
| High = 15 | Highly active renal involvement, such as glomerular involvement with proteinuria >1.5 g/day or hematuria or renal failure (GFR <60 mL/minute), or histologic evidence of proliferative glomerulonephritis or cryoglobulinemia-related renal involvement | ||
| Muscular | Exclusion of weakness due to glucocorticoids | No = 0 | Absence of currently active muscular involvement |
| Low = 6 | Mild active myositis shown by abnormal EMG, MRI, or biopsy with no weakness and creatine kinase (CK ≥ULN and ≤2x ULN) | ||
| Moderate = 12 | Moderately active myositis confirmed by abnormal EMG, MRI, or biopsy with weakness (maximal deficit of 4/5) or elevated creatine kinase (CK >2x ULN and ≤4x ULN) | ||
| High = 18 | Highly active myositis shown by abnormal EMG, MRI, or biopsy with weakness (deficit ≤3/5) or elevated creatine kinase (>4x ULN) | ||
| 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 PNS involvement, such as pure sensory axonal polyneuropathy shown by NCS or trigeminal (V) neuralgia Proven small fiber neuropathy | ||
| Moderate = 10 | Moderately active PNS involvement shown by NCS, such as axonal sensorimotor 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 4/5 or 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 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 | 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 confirmed cognitive impairment | ||
| High = 15 | Highly active CNS features, such as cerebral vasculitis with cerebrovascular accident or transient ischemic attack, seizures, transverse myelitis, lymphocytic meningitis, multiple sclerosis-like symptoms with motor deficit | ||
| Hematologic | For anemia, neutropenia, and thrombocytopenia, 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 (ANC >1000 and <1500/mm3) and/or anemia (hemoglobin >10 and <12 g/dL) and/or thrombocytopenia (platelets >100,000 and <150,000/mm3) or Lymphopenia (lymphocytes >500 and <1000/mm3) | ||
| Moderate = 4 | Cytopenia of autoimmune origin with neutropenia (ANC ≥500 and ≤1000/mm3) and/or anemia (hemoglobin ≥8 and ≤10 g/dL) and/or thrombocytopenia (platelets ≥50,000 and ≤100,000/mm3) or Lymphopenia (lymphocytes ≤500/mm3) | ||
| High = 6 | Cytopenia of autoimmune origin with neutropenia (ANC <500/mm3) and/or anemia (hemoglobin <8 g/dL) and/or thrombocytopenia (platelets <50,000/mm3) | ||
| Biologic | No = 0 | Absence of any of the following biologic 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) |
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