ICD-10-CM · General

M35.1

M35.1 classifies other overlap syndromes, including mixed connective tissue disease (MCTD) — an autoimmune condition combining clinical features of systemic lupus erythematosus, systemic sclerosis, polymyositis, and Raynaud's phenomenon, typically confirmed by positive anti-U1 RNP antibodies.

Verified May 8, 2026 · 5 sources ↓

Status
Billable
Chapter
13
Related CPT
5
Region
General
Drawn from CDCCMSicd10data.com 2026AAPCMS

Documentation tips

What should appear in the chart to support M35.1.

Source · Editorial brief grounded in 5 cited references ↓

  • Document the specific overlap syndrome by name — 'mixed connective tissue disease' or the component conditions overlapping (e.g., SLE + scleroderma features) — not just 'overlap syndrome NOS'.
  • Record serologic findings explicitly: positive anti-U1 RNP antibody titer supports MCTD and justifies M35.1 over the unspecified M35.9.
  • List each active clinical overlap feature present at the encounter (Raynaud's phenomenon, synovitis, esophageal dysmotility, myopathy) to support medical necessity and distinguish from a single-disease diagnosis.
  • If a rheumatologist's outside note establishes the MCTD diagnosis, reference it explicitly in the encounter note so the coder can confirm M35.1 is appropriate rather than defaulting to M35.9.
  • Note any Excludes1 conditions being managed separately (e.g., polyangiitis overlap syndrome under M30.8) to ensure they are coded independently and not under M35.1.

Related CPT procedures

Procedure codes commonly billed with M35.1. Linking the right diagnosis to the right procedure is what establishes medical necessity.

Source · CMS LCDs · AAOS specialty guidance · claims-pattern analysis

Common coding pitfalls

The recurring mistakes coders make with M35.1 and adjacent codes.

Source · Editorial brief grounded in CDC ICD-10-CM tabular guidance, AAOS coding references, and cited references ↓

  • Coding M35.1 when the provider documented only 'connective tissue disease' without specifying an overlap syndrome or MCTD — that defaults to M35.9, not M35.1.
  • Assigning M35.1 alongside M30.8 for polyangiitis overlap syndrome: the Excludes1 note at M35 prohibits using both codes for the same encounter.
  • Using M35.1 for a single defined autoimmune diagnosis (e.g., pure SLE or pure systemic sclerosis) — overlap coding requires documented features of two or more distinct connective tissue diseases.
  • Omitting additional codes for significant manifestations (e.g., Raynaud's phenomenon, interstitial lung disease, inflammatory myopathy) that are separately reportable and affect DRG severity and reimbursement.

Clinical context

Source · Editorial summary grounded in 5 cited references ↓

Use M35.1 when the provider has documented an overlap syndrome — most commonly mixed connective tissue disease (MCTD) — characterized by serologic evidence of anti-U1 RNP antibodies alongside clinical features drawn from two or more of the following: SLE, scleroderma, polymyositis, and Raynaud's phenomenon. The code also captures mixed collagen vascular disease. Do not use M35.1 for polyangiitis overlap syndrome; that maps to M30.8 (Excludes1 at the M35 parent level).

In orthopedic and rheumatology practice, MCTD patients frequently present with joint complaints — synovitis, polyarthralgia, or hand swelling — that can trigger referral to an orthopedic or hand surgeon before a definitive connective tissue diagnosis is established. Once the rheumatologist's MCTD diagnosis is documented in the record, M35.1 becomes the appropriate primary or secondary diagnosis code for the encounter.

If the provider documents only generic 'autoimmune disease' or 'connective tissue disorder' without specifying MCTD or a named overlap syndrome, drop to M35.9 (systemic involvement of connective tissue, unspecified). M35.1 requires that the specific overlap syndrome or MCTD be named or clearly implied by documented serologic and clinical criteria. MS-DRG v43.0 groups M35.1 under DRGs 545–547 (Connective tissue disorders with/without CC/MCC).

Inclusion & exclusion notes

Per the official ICD-10-CM Tabular List.

Source · CDC ICD-10-CM Official Tabular List · 2026

Includes

  • Mixed connective tissue disease

Excludes 1 — never code together

  • polyangiitis overlap syndrome (M30.8)

Sibling codes

Other billable codes under M35 (laterality / anatomic variants).

Frequently asked questions

Source · Generated from the editorial pipeline, verified against 5 cited references ↓

01What is the difference between M35.1 and M35.9?
M35.1 requires documentation of a specific overlap syndrome — most often MCTD with named component diseases and/or positive anti-U1 RNP antibody. M35.9 is the fallback when the provider documents systemic connective tissue involvement without naming the specific disorder. Never assign M35.1 solely on the basis of vague autoimmune symptoms.
02Can M35.1 be used for polyangiitis overlap syndrome?
No. Polyangiitis overlap syndrome is coded to M30.8. An Excludes1 note at the M35 parent level prohibits assigning M35.1 and M30.8 together — these two conditions are mutually exclusive under ICD-10-CM convention.
03Does M35.1 cover both MCTD and other overlap syndromes, or only MCTD?
M35.1 covers all overlap syndromes in this category, with MCTD listed as the primary 'Applicable To' example. Other named overlap presentations combining features of SLE, scleroderma, and polymyositis also map here when documented by the provider as an overlap syndrome.
04Should I code the individual component conditions (SLE, scleroderma, myopathy) separately in addition to M35.1?
Not for the overlap syndrome itself — M35.1 captures the combined diagnosis. However, distinct manifestations that are separately managed (e.g., interstitial lung disease, inflammatory arthritis, Raynaud's phenomenon) may be coded additionally when clinically documented and addressed at the encounter.
05What MS-DRG does M35.1 fall under for inpatient claims?
Under MS-DRG v43.0, M35.1 groups to DRG 545 (Connective tissue disorders with MCC), 546 (with CC), or 547 (without CC/MCC), depending on the patient's complication and comorbidity profile. Coding all documented manifestations accurately determines which severity tier applies.
06Is serology required to assign M35.1, or can the provider's clinical diagnosis alone support the code?
The provider's documented diagnosis controls code assignment — if the physician states MCTD or overlap syndrome, M35.1 is appropriate. However, recording the positive anti-U1 RNP antibody in the note strengthens the record against audit challenge and distinguishes MCTD from other overlap presentations.

Sources & references

Editorial content was developed using the following public sources. Last verified May 8, 2026.

  1. 01CDC ICD-10-CM Tabular List 2026 (effective October 1, 2025)
  2. 02CMS ICD-10-CM Official Guidelines for Coding and Reporting FY 2026 — https://www.cms.gov/files/document/fy-2026-icd-10-cm-coding-guidelines.pdf
  3. 03icd10data.com 2026 ICD-10-CM Diagnosis Code M35.1 — https://www.icd10data.com/ICD10CM/Codes/M00-M99/M30-M36/M35-/M35.1
  4. 04AAPC Codify ICD-10 Code M35.1 — https://www.aapc.com/codes/icd-10-codes/M35.1
  5. 05MS-DRG v43.0 Grouper — CMS

Mira AI Scribe

Mira captures the provider's stated diagnosis of mixed connective tissue disease or named overlap syndrome, documented anti-U1 RNP antibody results, and each active clinical feature (Raynaud's, synovitis, myopathy, etc.) present at the encounter. This prevents downgrade to the nonspecific M35.9 and ensures the chart supports M35.1 at audit without the coder needing to chase rheumatology records.

See how Mira captures M35.1 documentation

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