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Scleroderma

 

Thank you for asking me to examine Harry, who presented with difficulty using his hands.

 

I would like to complete my exam by:

  • A full set of vital signs

  • Complete respiratory and cardiac examinations

 

My findings are in keeping with limited/diffuse scleroderma complicated by _____.

 

On general inspection, there was/was not scleroderma facies, and portable oxygen etc.

 

On examination of the hands there was a contraction deformity of the fingers with sclerodactyly extending to ______ with/without reduced hair loss in the arms. This was / was not associated with calcinosis, Raynaud’s, telangiectasia and digital tip ulceration and pitting, and atrophy of the distal pulp. There was coldness to touch. There was / was not features of active synovitis.

 

There was impaired / unaffected function of the hand with grip strength, pincer grip, opposition grip, and difficulty undoing a button and taking off a jar lid.

 

In terms of other features suggestive of scleroderma:

  • In the face there was:

    • Skin tethering in the face with reduced mouth aperture

    • Inability to close the eyes completely

    • Telangiectasia

    • Dry eyes and mouth

    • Alopecia

  • On the chest, there was thickened chest wall with skin tightening

  • In the legs, there was skin tethering in the legs with ulceration

 

In terms of complications of scleroderma:

  • There was/was not hypertension. There was / was not a fistula suggesting ESKD.

  • There was/was not proximal myopathy in the arms

  • There was/was not evidence of pulmonary hypertension

  • There was/was not evidence of pulmonary fibrosis

  • There was/was not evidence of cor pulmonale

  • There was/was not a PEG or stoma suggestive severe oesophageal dysmotility

 

In summary my findings are in keeping with limited/diffuse scleroderma with evidence of ILD/pulmonary HTN/HTN complications.

 

A differential diagnosis would be mixed connective tissue disease.

 

I would proceed from here by:

  • Confirming my diagnosis with an appropriate antibody panel including Scl70, RNA polymerase III, anti-centromere and anti u1 rnp (Anti-U1-ribonucleoprotein) antibodies.

  • I would confirm suspected complications and screen for others:

    • UEC, urine (bland) - renal

    • Pulmonary function tests, ECG, TTE - pulmonary hypertension

    • Pulmonary function tests, HRCT - ILD

    • GI investigations - GORD, dysmotility

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