Table 1.
Diagnostic criteria for mixed connective tissue disorder [15].
| Sharp | Major criteria | Minor criteria | Diagnosis |
| (1) Myositis (2) Pulmonary involvement: (a) Diffusion capacity < 70% of normal values (b) Pulmonary hypertension (c) Proliferative vascular lesions on lung biopsy (3) Raynaud's phenomenon or esophageal hypomotility (4) Swollen hands (5) Anti-ENA Ab N 1 : 10,000 and anti-U1 RNP Ab positive and anti-Sm negative |
(1) Alopecia (2) Leukopenia (3) Anemia (4) Pleuritis (5) Pericarditis (6) Arthritis (7) Trigeminal neuropathy (8) Malar rash (9) Thrombocytopenia (10) Mild myositis (11) History of swollen hands |
At least 4 major criteria plus anti-U1-RNP Ab titer of at least 1 : 4000 or two major criteria from among criteria 1, 2, and 3 plus 2 minor criteria plus anti-U1-RNP Ab titer of at least 1 : 1000 Exclusion criteria: positivity for anti-Sm Ab |
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| Kasukawa | Common symptoms | Mixed symptoms | Diagnosis |
| (1) Raynaud's phenomenon (2) Swollen fingers or hands anti-RNP Ab positive |
(1) SLE-like symptoms: (a) Polyarthritis (b) Lymphadenopathy (c) Facial erythema (d) Pericarditis or pleuritis (e) Leukopenia or thrombocytopenia. (2) SSc-like findings: (a) Sclerodactyly (b) Pulmonary fibrosis, restrictive changes of lung, or reduced diffusion capacity (c) Hypomotility or dilatation of esophagus. (3) PM-like findings: (a) Muscle weakness (b) Elevated serum levels of muscle enzymes (CPK) (c) Myogenic pattern on EMG |
At least one of common symptoms plus positivity for anti-RNP Ab plus one or more signs/symptoms of the mixed symptoms in at least two of the three disease categories | |
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| Alarcón-Segovia | Serological criteria | Clinical criteria | Diagnosis |
| Anti-RNP Ab titer N 1 : 1000 | (1) Edema in hands (2) Synovitis (3) Myositis (4) Raynaud's phenomenon (5) Acrosclerosis |
Serological criteria plus at least 3 clinical criteria included either synovitis or myositis | |
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| Kahn | Serological criteria | Clinical criteria | Diagnosis |
| Presence of high titer anti-RNP Ab corresponding to speckled ANA at titer ≥ 1 : 2000 |
(1) Raynaud's phenomenon (2) Synovitis (3) Myositis (4) Swollen fingers |
Serological criteria plus Raynaud's phenomenon and at least two of the three following signs (synovitis, myositis, and swollen fingers) | |
Abbreviations: RNP: ribonucleoprotein; ENA: extractable nuclear antigen; Sm: Smith; SLE: systemic lupus erythematosus; SSc: systemic sclerosis; PM: polymyositis; CPK: creatine phosphokinase; EMG: electromyography; ANA: anti-nuclear antigen.