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Scleroderma

Ann M. Lynch, AE-C, RPh, PharmD, BS Pharmacy and Jeremy Golding, FAAFP, MD Reviewed 05/2023
 


BASICS

DESCRIPTION

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

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 Rh...

ADDITIONAL READING

SEE ALSO

  • Fernández-Codina A, Walker KM, Pope JE, et al.; Scleroderma Algorithm Group. Treatment Algorithms for Systemic Sclerosis According to Experts. Arthritis Rheuma...

SEE ALSO

Morphea 

CODES

ICD10

  • M34.2 Systemic sclerosis induced by drug and chemical

  • M34.9 Systemic sclerosis, unspecified

  • M34.0 Progressive systemic sclerosis

  • L94.0 Localized scleroderma [morphea]

  • M34 Systemic sclerosis [sc...

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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