Description

Kusukawa et al developed preliminary criteria for the diagnosis of mixed connective tissue disease (MCTD). The authors are from the Mixed Connective Tissue Disease Research Committee in Japan.


 

Parameters:

(1) common symptoms (2)

(2) antibody to nRNP

(3) mixed finding groups

(3a) SLE-like findings

(3b) PSS-like findings

(3c) polymyositis (PM) like findings

 

Criteria for the diagnosis of MCTD:

(1) one or both of the common symptoms

(2) presence of anti-nRNP antibodies

(3) presence of >= 1 findings in >= 2 of the mixed finding groups

 

Common symptoms:

(1) Raynaud's phenomenon

(2) swollen fingers or hands

 

SLE-like findings:

(1) polyarthritis

(2) lymphadenopathy

(3) facial erythema

(4) pericarditis OR pleuritis

(5) leukopenia OR thrombocytopenia

 

PSS-like findings:

(1) sclerodactyly

(2) pulmonary fibrosis OR restrictive pattern OR reduced diffusion capacity

(3) hypomotility OR dilatation of esophagus

 

PM-like findings:

(1) muscle weakness

(2) increased serum CK concentration

(3) myogenic pattern on EMG

 

where:

• Leukopenia is a WBC count < 4,000 per µL.

• Thrombocytopenia is a platelet count < 100,000 per µL.

• A restrictive pattern in pulmonary function is indicated by a vital capacity < 80% of predicted.

• A reduced diffusion capacity is indicated by a CO diffusing capacity < 70% of predicted.

 


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