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Scleroderma

Ann M. Lynch, PharmD, RPh, AE-C and Deborah M. DeMarco, MD, FACP Reviewed 06/2018
 


BASICS

DESCRIPTION

  • Scleroderma (systemic sclerosis [SSc]) is a chronic disease of unknown cause characterized by diffuse fibrosis of skin and visceral organs and vascular abnormalities.

  • Most manifestati...

DIAGNOSIS

HISTORY

  • Raynaud phenomenon is generally the presenting complaint (differentiated from Raynaud disease, generally affecting younger individuals and without digital ulcers).

  • Skin thickening, “pu...

TREATMENT

GENERAL MEASURES

  • Treatment is symptomatic and supportive.

  • Esophageal dilation may be used for strictures.

  • Avoid cold; dress appropriately in layers for the weather; be wary of air conditioning.

  • ...

ONGOING CARE

FOLLOW UP RECOMMENDATIONS

Patient Monitoring

  • Monitor every 3 to 6 months for end-organ and skin involvement and medications. Provide encouragement.

  • Echocardiology and PFTs yearly

DIET

Drink pl...

REFERENCES

1
van den Hoogen F, Khanna D, Fransen J, et al. 2013 Classification criteria for systemic sclerosis: an American College of Rheumatology/European League Against Rheumatism collaborative init...

ADDITIONAL READING

  • Herrick AL. Contemporary management of Raynaud’s phenomenon and digits ischaemic complications. Curr Opin Rheumatol.  2011;23(6):555–561. [View Abstract on OvidMedline]

  • Kowal-B...

SEE ALSO

Morphea 

CODES

ICD10

  • M34.9 Systemic sclerosis, unspecified

  • M34.1 CR(E)ST syndrome

  • L94.0 Localized scleroderma [morphea]

  • L94.1 Linear scleroderma

  • M34.89 Other systemic sclerosis

  • M34.83 Systemic sclerosis with polyneu...

CLINICAL PEARLS

  • Raynaud phenomenon is frequently the initial complaint.

  • Skin thickening, “puffy hands,” and GERD are often noted early in disease.

  • Patients must be followed proactively for development o...

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