M34.1

Billable

CR(E)ST syndrome

HCC Category Mapping

V28HCC 93 — Rheumatoid Arthritis and Inflammatory Connective Tissue Disease

0.175

V24HCC 40 — Rheumatoid Arthritis and Inflammatory Connective Tissue Disease

0.307

ESRDHCC 40 — Rheumatoid Arthritis and Inflammatory Connective Tissue Disease

0.000

RxHCCHCC 82 — Systemic Sclerosis (Scleroderma)

0.000

What This Code Means

A limited form of systemic sclerosis characterized by calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia.

Coding Tips

•CREST is an acronym for the five main features; document which features are present
•This represents a more limited form of systemic sclerosis with generally better prognosis than diffuse disease

Clinical Significance

CREST syndrome represents a limited form of systemic sclerosis with specific clinical features but generally better prognosis than diffuse disease. However, it still carries risks for pulmonary hypertension and other complications requiring ongoing monitoring and specialized care.

Documentation Requirements

✓Documentation of CREST syndrome diagnosis
✓Evidence of characteristic features (calcinosis, Raynaud's, esophageal dysmotility, sclerodactyly, telangiectasia)
✓Anticentromere antibody testing results
✓Assessment for pulmonary hypertension
✓Gastrointestinal evaluation including swallowing studies
✓Skin involvement pattern documentation
✓Treatment plan addressing specific manifestations
✓Monitoring plan for complications

Commonly Confused Codes

M34.0 — Use for progressive systemic sclerosis (diffuse form)M34.9 — Use for unspecified systemic sclerosis I27.0 — Use additionally for pulmonary hypertension when present K22.0 — Use additionally for esophageal involvement

Code Hierarchy

└M34Systemic sclerosis [scleroderma]└M34.1CR(E)ST syndrome

└M34.1CR(E)ST syndrome