Clinical and Immunological Profile of Mixed Connective Tissue Disease and a Comparison of Four Diagnostic Criteria

Table 1

Diagnostic criteria for mixed connective tissue disorder [15].

Sharp	Major criteria	Minor criteria	Diagnosis
	(1) Myositis; (2) Pulmonary involvement: (a) Diffusion 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
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
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
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.