International Journal of Rheumatology / 2020 / Article / Tab 1

Research Article

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].

SharpMajor criteriaMinor criteriaDiagnosis
(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

KasukawaCommon symptomsMixed symptomsDiagnosis
(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-SegoviaSerological criteriaClinical criteriaDiagnosis
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

KahnSerological criteriaClinical criteriaDiagnosis
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.

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