M30-M36
Medium Complexity

Systemic connective tissue disorders

Primary Specialty: Rheumatology
Last Updated: 2025-09-10

ICD-10 Codes (92)

76 billable
10 category headers
M31
Other necrotizing vasculopathies
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M31.0
Billable
Hypersensitivity angiitis
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M31.1
Thrombotic microangiopathy
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M31.10
Billable
Thrombotic microangiopathy, unspecified
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M31.11
Billable
Hematopoietic stem cell transplantation-associated thrombotic microangiopathy [HSCT-TMA]
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M31.19
Billable
Other thrombotic microangiopathy
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M31.2
Billable
Lethal midline granuloma
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M31.3
Wegener's granulomatosis
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M31.30
Billable
Wegener's granulomatosis without renal involvement
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M31.31
Billable
Wegener's granulomatosis with renal involvement
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M31.4
Billable
Aortic arch syndrome [Takayasu]
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M31.5
Billable
Giant cell arteritis with polymyalgia rheumatica
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M31.6
Billable
Other giant cell arteritis
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M31.7
Billable
Microscopic polyangiitis
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M31.8
Billable
Other specified necrotizing vasculopathies
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M31.9
Billable
Necrotizing vasculopathy, unspecified
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M32
Systemic lupus erythematosus (SLE)
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M32.0
Billable
Drug-induced systemic lupus erythematosus
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M32.1
Systemic lupus erythematosus with organ or system involvement
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M32.10
Billable
Systemic lupus erythematosus, organ or system involvement unspecified
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M32.11
Billable
Endocarditis in systemic lupus erythematosus
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M32.12
Billable
Pericarditis in systemic lupus erythematosus
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M32.13
Billable
Lung involvement in systemic lupus erythematosus
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M32.14
Billable
Glomerular disease in systemic lupus erythematosus
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M32.15
Billable
Tubulo-interstitial nephropathy in systemic lupus erythematosus
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M32.19
Billable
Other organ or system involvement in systemic lupus erythematosus
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M32.8
Billable
Other forms of systemic lupus erythematosus
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M32.9
Billable
Systemic lupus erythematosus, unspecified
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M33
Dermatopolymyositis
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M33.0
Juvenile dermatomyositis
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M33.00
Billable
Juvenile dermatomyositis, organ involvement unspecified
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M33.01
Billable
Juvenile dermatomyositis with respiratory involvement
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M33.02
Billable
Juvenile dermatomyositis with myopathy
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M33.03
Billable
Juvenile dermatomyositis without myopathy
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M33.09
Billable
Juvenile dermatomyositis with other organ involvement
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M33.1
Other dermatomyositis
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M33.10
Billable
Other dermatomyositis, organ involvement unspecified
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M33.11
Billable
Other dermatomyositis with respiratory involvement
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M33.12
Billable
Other dermatomyositis with myopathy
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M33.13
Billable
Other dermatomyositis without myopathy
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M33.19
Billable
Other dermatomyositis with other organ involvement
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M33.2
Polymyositis
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M33.20
Billable
Polymyositis, organ involvement unspecified
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M33.21
Billable
Polymyositis with respiratory involvement
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M33.22
Billable
Polymyositis with myopathy
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M33.29
Billable
Polymyositis with other organ involvement
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M33.9
Dermatopolymyositis, unspecified
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M33.90
Billable
Dermatopolymyositis, unspecified, organ involvement unspecified
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M33.91
Billable
Dermatopolymyositis, unspecified with respiratory involvement
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M33.92
Billable
Dermatopolymyositis, unspecified with myopathy
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M33.93
Billable
Dermatopolymyositis, unspecified without myopathy
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M33.99
Billable
Dermatopolymyositis, unspecified with other organ involvement
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M34
Systemic sclerosis [scleroderma]
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M34.0
Billable
Progressive systemic sclerosis
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M34.1
Billable
CR(E)ST syndrome
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M34.2
Billable
Systemic sclerosis induced by drug and chemical
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M34.8
Other forms of systemic sclerosis
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M34.81
Billable
Systemic sclerosis with lung involvement
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M34.82
Billable
Systemic sclerosis with myopathy
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M34.83
Billable
Systemic sclerosis with polyneuropathy
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M34.89
Billable
Other systemic sclerosis
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M34.9
Billable
Systemic sclerosis, unspecified
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M35
Other systemic involvement of connective tissue
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M35.0
Sjögren syndrome
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M35.00
Billable
Sjögren syndrome, unspecified
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M35.01
Billable
Sjögren syndrome with keratoconjunctivitis
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M35.02
Billable
Sjögren syndrome with lung involvement
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M35.03
Billable
Sjögren syndrome with myopathy
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M35.04
Billable
Sjögren syndrome with tubulo-interstitial nephropathy
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M35.05
Billable
Sjögren syndrome with inflammatory arthritis
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M35.06
Billable
Sjögren syndrome with peripheral nervous system involvement
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M35.07
Billable
Sjögren syndrome with central nervous system involvement
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M35.08
Billable
Sjögren syndrome with gastrointestinal involvement
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M35.09
Billable
Sjögren syndrome with other organ involvement
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M35.1
Billable
Other overlap syndromes
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M35.2
Billable
Behçet's disease
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M35.3
Billable
Polymyalgia rheumatica
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M35.4
Billable
Diffuse (eosinophilic) fasciitis
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M35.5
Billable
Multifocal fibrosclerosis
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M35.6
Billable
Relapsing panniculitis [Weber-Christian]
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M35.7
Billable
Hypermobility syndrome
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M35.8
Other specified systemic involvement of connective tissue
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M35.81
Billable
Multisystem inflammatory syndrome
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M35.89
Billable
Other specified systemic involvement of connective tissue
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M35.9
Billable
Systemic involvement of connective tissue, unspecified
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M36
Systemic disorders of connective tissue in diseases classified elsewhere
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M36.0
Billable
Dermato(poly)myositis in neoplastic disease
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M36.1
Billable
Arthropathy in neoplastic disease
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M36.2
Billable
Hemophilic arthropathy
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M36.3
Billable
Arthropathy in other blood disorders
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M36.4
Billable
Arthropathy in hypersensitivity reactions classified elsewhere
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M36.8
Billable
Systemic disorders of connective tissue in other diseases classified elsewhere
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Updates & Changes

FY 2026 Updates

Current Year

Deleted Codes

No codes deleted in this range for FY 2026

No significant changes for FY 2026

This range maintains stability with current coding practices

Historical Changes

  • FY 2025: Routine maintenance updates with minor terminology clarifications
  • FY 2024: Enhanced specificity requirements for certain code ranges
  • FY 2023: Updated documentation guidelines for improved clarity

Upcoming Changes

  • Proposed updates pending review by Coordination and Maintenance Committee
  • Under consideration: Enhanced digital health integration codes

Implementation Guidance

  • Review all FY 2026 updates for M30-M36 codes before implementation
  • Always verify the most current codes in the ICD-10-CM manual
  • Ensure clinical documentation supports the selected diagnosis codes
  • +3 more guidance items...

Range Overview

high priority

The ICD-10 code range M30-M36 pertains to systemic connective tissue disorders. These codes are used to classify a variety of conditions that affect the body's connective tissues, including vasculitis, polymyalgia rheumatica, and systemic lupus erythematosus. The codes are organized by specific disorder, with further subdivisions for manifestations and complications.

Key Usage Points:

  • Always code to the highest level of specificity, considering the type of disorder, the affected body system, and any associated complications.
  • Use additional codes to identify any associated conditions or manifestations not covered by the combination codes.
  • When coding for systemic lupus erythematosus, use M32.9 if no organ or system is involved.
  • For polymyalgia rheumatica, use M35.3. If giant cell arteritis is present, use M31.5 instead.
  • For vasculitis, use M30-M31. The specific code will depend on the type and location of the vasculitis.

Coding Guidelines

When to Use:

  • When a patient is diagnosed with a systemic connective tissue disorder.
  • When a patient with a known systemic connective tissue disorder presents with a new manifestation or complication.
  • When a patient's systemic connective tissue disorder is a significant factor in their current medical management.
  • When a patient has a history of a systemic connective tissue disorder that affects their current health status.

When NOT to Use:

  • When a patient has a localized connective tissue disorder. Use codes from M00-M25 instead.
  • When a patient has a connective tissue disorder that is secondary to another condition. Use the code for the primary condition instead.
  • When a patient has a connective tissue disorder that is not systemic. Use the appropriate code from M00-M99.
  • When a patient has a history of a systemic connective tissue disorder that does not affect their current health status. Use a code from Z80-Z87 instead.

Code Exclusions

Always verify exclusions in the Tabular List of the ICD-10-CM.

Documentation Requirements

Documentation for systemic connective tissue disorders should include the specific diagnosis, the affected body system(s), and any associated complications or manifestations. If the disorder is a significant factor in the patient's current medical management, this should also be documented.

Clinical Information:

  • Specific diagnosis
  • Affected body system(s)
  • Associated complications or manifestations
  • Impact on current medical management

Supporting Evidence:

  • Clinical notes
  • Laboratory and imaging results
  • Referral letters
  • Hospital admission and discharge summaries
Good Documentation Example:

Patient diagnosed with systemic lupus erythematosus with renal involvement. The disease is currently active and is a significant factor in the patient's medical management.

Poor Documentation Example:

Patient has lupus.

Common Documentation Errors:

  • Not documenting the specific type of disorder
  • Not documenting the affected body system(s)
  • Not documenting associated complications or manifestations
  • Not documenting the impact on current medical management

Range Statistics

7
Total Codes
76
Billable
Complexity:
Medium
Primary Use:Clinical Documentation
Chapter:13

Coding Complexity

Medium
Complexity Rating

Coding for systemic connective tissue disorders is of medium complexity due to the need to understand the specific types of disorders, identify the affected body system(s), and recognize associated complications or manifestations. The impact on current medical management must also be considered.

Key Factors:
  • Understanding the specific types of systemic connective tissue disorders
  • Identifying the affected body system(s)
  • Identifying associated complications or manifestations
  • Determining the impact on current medical management

Specialty Focus

The M30-M36 range is primarily used by rheumatologists, but may also be used by other specialists such as nephrologists and cardiologists, depending on the affected body system.

Primary Specialties:
Rheumatology
60%
Nephrology
20%
Cardiology
20%
Clinical Scenarios:
  • A patient with systemic lupus erythematosus presents with new-onset renal failure.
  • A patient with polymyalgia rheumatica develops giant cell arteritis.
  • A patient with a known systemic connective tissue disorder presents with a new cardiac manifestation.
  • A patient's systemic connective tissue disorder is a significant factor in their current medical management.
  • A patient has a history of a systemic connective tissue disorder that affects their current health status.

Resources & References

Resources for coding systemic connective tissue disorders include the ICD-10-CM Official Guidelines for Coding and Reporting, the American College of Rheumatology's guidelines, and various clinical reference books and online resources.

Official Guidelines:

  • ICD-10-CM Official Guidelines for Coding and Reporting
  • American College of Rheumatology guidelines
  • World Health Organization's International Classification of Diseases

Clinical References:

  • Harrison's Principles of Internal Medicine
  • UpToDate

Educational Materials:

  • American Health Information Management Association (AHIMA) resources
  • American Academy of Professional Coders (AAPC) resources

Frequently Asked Questions

Can I use a code from the M30-M36 range for a patient with a localized connective tissue disorder?

No, the M30-M36 range is for systemic connective tissue disorders. For localized connective tissue disorders, use a code from the M00-M25 range.