Systemic sclerosis [scleroderma] (M34)
ICD-10 codes M34 and its subcategories specifically classify systemic sclerosis, commonly known as scleroderma, a complex autoimmune condition that causes skin thickening and organ fibrosis. These codes are essential for identifying the various forms and complications of systemic sclerosis in medical records and billing.
The section includes the base code M34 for systemic sclerosis and detailed codes such as M34.0 for progressive systemic sclerosis, and M34.1 for CREST syndrome; also referred to as Raynaud's phenomenon, limited systemic sclerosis, or CREST syndrome; highlighting its limited skin involvement. Other codes cover systemic sclerosis caused by drugs or chemicals (M34.2) and subtypes with specific organ involvement like lung disease (M34.81), myopathy (M34.82), or polyneuropathy (M34.83). The codes M34.8 and M34.89 address other systemic sclerosis variants, including pediatric or diffuse forms, while M34.9 is used for unspecified systemic sclerosis with a broad range of related complications. Using the ICD-10 code for systemic sclerosis accurately helps clinicians and coders capture the precise type and associated conditions, improving diagnosis and treatment documentation.
Diseases of the musculoskeletal system and connective tissue (M00–M99)
Systemic connective tissue disorders (M30-M36)
M34 Systemic sclerosis [scleroderma]
- M34.0 Progressive systemic sclerosis
- M34.1 CR(E)ST syndrome
- M34.2 Systemic sclerosis induced by drug and chemical
M34.8 Other forms of systemic sclerosis
- M34.81 Systemic sclerosis with lung involvement
- M34.82 Systemic sclerosis with myopathy
- M34.83 Systemic sclerosis with polyneuropathy
- M34.89 Other systemic sclerosis
- M34.9 Systemic sclerosis, unspecified
Systemic sclerosis [scleroderma] (M34)
Instructional Notations
Type 1 Excludes
A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
Clinical Terms
The following clinical terms provide additional context, helping users better understand the clinical background and common associations for each diagnosis listed in this section. Including related terms alongside ICD-10-CM codes supports coders, billers, and healthcare professionals in improving accuracy, enhancing documentation, and facilitating research or patient education.
CREST Syndrome
A mild form of LIMITED SCLERODERMA, a multi-system disorder. Its features include symptoms of CALCINOSIS; RAYNAUD DISEASE; ESOPHAGEAL MOTILITY DISORDERS; sclerodactyly, and TELANGIECTASIS. When the defect in esophageal function is not prominent, it is known as CRST syndrome.
