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Chapter: Paediatrics: Bones and joints

Paediatrics: Mixed connective tissue disease - overlap syndromes

Combined features of SLE, progressive systemic sclerosis, and dermato-myostis with positive ribonucleoprotein (RNP) antibody.

Mixed connective tissue disease— overlap syndromes

 

Combined features of SLE, progressive systemic sclerosis, and dermato-myostis with positive ribonucleoprotein (RNP) antibody.

   Prognosis and management similar to that of SLE , but more benign prognosis because renal and CNS involvement are rare.

 

Clinical features

 

   Raynaud’s phenomenon (common).

   Swollen hands and fingers (common).

   Polyarthritis: symmetrical peripheral.

   Rashes: similar to SLE or JDM rash; tight non-elastic skin of hands.

   Muscle weakness and myositis.

   Restrictive lung disease and pulmonary hypertension (rare).

 

Differential diagnosis

 

   Systemic onset JIA.

 

   RF-positive polyarticular JIA.

 

   SLE.

 

Investigations

 

   Characteristic anti-RNP antibody.

 

   Other autoantibodies: ANA +ve (90%); RF +ve (often).

 

   FBC: leucopenia; thrombocytopenia.

 

   CXR.

 

·  Renal function.

 

Echocardiogram: screen for right ventricular hypertrophy secondary to pulmonary hypertension in established disease.

 

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Paediatrics: Bones and joints


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