M30.3

Billable

Mucocutaneous lymph node syndrome [Kawasaki]

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 84 — Vasculitis and Other Autoimmune Disorders

0.000

What This Code Means

Kawasaki disease is an acute inflammatory condition primarily affecting young children, causing fever, rash, and inflammation of blood vessels that can lead to heart complications.

Coding Tips

•This is typically an acute condition in children; document the phase of illness (acute, subacute, or convalescent)
•Always screen for cardiac complications as coronary artery involvement is a serious sequela

Clinical Significance

Kawasaki disease is an acute febrile vasculitis primarily affecting children under 5 years, with potential for serious cardiac complications including coronary artery aneurysms. Early recognition and treatment with IVIG are critical to prevent long-term cardiovascular sequelae.

Documentation Requirements

✓Patient age typically under 5 years
✓Fever lasting at least 5 days
✓At least 4 of 5 clinical criteria (rash, conjunctivitis, lymphadenopathy, extremity changes, oral changes)
✓Echocardiogram results documenting coronary artery status
✓Laboratory evidence of inflammation
✓Response to IVIG treatment if administered
✓Long-term cardiac monitoring plan
✓Documentation excluding other febrile illnesses

Commonly Confused Codes

M30.2 — Juvenile polyarteritis (different pediatric vasculitis)A49.9 — Bacterial infection, unspecified (when fever is primary focus)B09 — Unspecified viral infection (when viral etiology suspected)M35.8 — Other specified systemic involvement of connective tissue R50.9 — Fever, unspecified (when only febrile component coded)

Code Hierarchy

└M30Polyarteritis nodosa and related conditions └M30.3Mucocutaneous lymph node syndrome [Kawasaki]

└M30.3Mucocutaneous lymph node syndrome [Kawasaki]