Systemic connective tissue disorders (M30-M36)

ICD-10 codes M30-M36 cover systemic connective tissue disorders, which include a variety of autoimmune and inflammatory diseases affecting multiple organs and tissues, such as polyarteritis nodosa, systemic lupus erythematosus, dermatomyositis, systemic sclerosis, and Sjogren syndrome.

This range specifically codes for complex connective tissue disorders characterized by inflammation and necrosis of blood vessels (vasculitis), muscle inflammation (myopathy), and involvement of organs like lungs, kidneys, and skin. For instance, M30.0 identifies polyarteritis nodosa and related medium-sized vessel vasculitis conditions, while M32. covers systemic lupus erythematosus (SLE), including drug-induced forms and organ-specific complications such as lupus nephritis and lung involvement. Dermatomyositis and polymyositis fall under M33, detailing juvenile and adult forms with or without muscle weakness. Systemic sclerosis (scleroderma), noted in M34, includes subtypes like CREST syndrome and drug-induced cases. Other disorders in this chapter like M35.0 describe Sjogren syndrome in various organ involvements, and M35.2 codes Behcet's disease, a multisystem inflammatory disorder. These codes aid in precise classification of connective tissue diseases based on clinical presentation and organ system involvement to guide treatment and research.

Polyarteritis nodosa and related conditions (M30)
Other necrotizing vasculopathies (M31)
Systemic lupus erythematosus (SLE) (M32)
Dermatopolymyositis (M33)
Systemic sclerosis [scleroderma] (M34)
Other systemic involvement of connective tissue (M35)
Systemic disorders of connective tissue in diseases classifi

Filter diagnosis codes list:

Diseases of the musculoskeletal system and connective tissue (M00–M99)
- Systemic connective tissue disorders (M30-M36)
  - - M30 Polyarteritis nodosa and related conditions
    - M30.0 Polyarteritis nodosa
    - M30.1 Polyarteritis with lung involvement [Churg-Strauss]
    - M30.2 Juvenile polyarteritis
    - M30.3 Mucocutaneous lymph node syndrome [Kawasaki]
    - M30.8 Other conditions related to polyarteritis nodosa
  - - M31 Other necrotizing vasculopathies
    - M31.0 Hypersensitivity angiitis
    - M31.1 Thrombotic microangiopathy
    - M31.10 Thrombotic microangiopathy, unspecified
    - M31.11 Hematopoietic stem cell transplantation-associated thrombotic microangiopathy [HSCT-TMA]
    - M31.19 Other thrombotic microangiopathy
    - M31.2 Lethal midline granuloma
    - M31.3 Wegener's granulomatosis
    - M31.30 Wegener's granulomatosis without renal involvement
    - M31.31 Wegener's granulomatosis with renal involvement
    - M31.4 Aortic arch syndrome [Takayasu]
    - M31.5 Giant cell arteritis with polymyalgia rheumatica
    - M31.6 Other giant cell arteritis
    - M31.7 Microscopic polyangiitis
    - M31.8 Other specified necrotizing vasculopathies
    - M31.9 Necrotizing vasculopathy, unspecified
  - - M32 Systemic lupus erythematosus (SLE)
    - M32.0 Drug-induced systemic lupus erythematosus
    - M32.1 Systemic lupus erythematosus with organ or system involvement
    - M32.10 Systemic lupus erythematosus, organ or system involvement unspecified
    - M32.11 Endocarditis in systemic lupus erythematosus
    - M32.12 Pericarditis in systemic lupus erythematosus
    - M32.13 Lung involvement in systemic lupus erythematosus
    - M32.14 Glomerular disease in systemic lupus erythematosus
    - M32.15 Tubulo-interstitial nephropathy in systemic lupus erythematosus
    - M32.19 Other organ or system involvement in systemic lupus erythematosus
    - M32.8 Other forms of systemic lupus erythematosus
    - M32.9 Systemic lupus erythematosus, unspecified
  - - M33 Dermatopolymyositis
    - M33.0 Juvenile dermatomyositis
    - M33.00 Juvenile dermatomyositis, organ involvement unspecified
    - M33.01 Juvenile dermatomyositis with respiratory involvement
    - M33.02 Juvenile dermatomyositis with myopathy
    - M33.03 Juvenile dermatomyositis without myopathy
    - M33.09 Juvenile dermatomyositis with other organ involvement
    - M33.1 Other dermatomyositis
    - M33.10 Other dermatomyositis, organ involvement unspecified
    - M33.11 Other dermatomyositis with respiratory involvement
    - M33.12 Other dermatomyositis with myopathy
    - M33.13 Other dermatomyositis without myopathy
    - M33.19 Other dermatomyositis with other organ involvement
    - M33.2 Polymyositis
    - M33.20 Polymyositis, organ involvement unspecified
    - M33.21 Polymyositis with respiratory involvement
    - M33.22 Polymyositis with myopathy
    - M33.29 Polymyositis with other organ involvement
    - M33.9 Dermatopolymyositis, unspecified
    - M33.90 Dermatopolymyositis, unspecified, organ involvement unspecified
    - M33.91 Dermatopolymyositis, unspecified with respiratory involvement
    - M33.92 Dermatopolymyositis, unspecified with myopathy
    - M33.93 Dermatopolymyositis, unspecified without myopathy
    - M33.99 Dermatopolymyositis, unspecified with other organ involvement
  - - 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
  - - M35 Other systemic involvement of connective tissue
    - M35.0 Sjogren syndrome
    - M35.00 Sjogren syndrome, unspecified
    - M35.01 Sjogren syndrome with keratoconjunctivitis
    - M35.02 Sjogren syndrome with lung involvement
    - M35.03 Sjogren syndrome with myopathy
    - M35.04 Sjogren syndrome with tubulo-interstitial nephropathy
    - M35.05 Sjogren syndrome with inflammatory arthritis
    - M35.06 Sjogren syndrome with peripheral nervous system involvement
    - M35.07 Sjogren syndrome with central nervous system involvement
    - M35.08 Sjogren syndrome with gastrointestinal involvement
    - M35.09 Sjogren syndrome with other organ involvement
    - M35.0A Sjogren syndrome with glomerular disease
    - M35.0B Sjogren syndrome with vasculitis
    - M35.0C Sjogren syndrome with dental involvement
    - M35.1 Other overlap syndromes
    - M35.2 Behcet's disease
    - M35.3 Polymyalgia rheumatica
    - M35.4 Diffuse (eosinophilic) fasciitis
    - M35.5 Multifocal fibrosclerosis
    - M35.6 Relapsing panniculitis [Weber-Christian]
    - M35.7 Hypermobility syndrome
    - M35.8 Other specified systemic involvement of connective tissue
    - M35.81 Multisystem inflammatory syndrome
    - M35.89 Other specified systemic involvement of connective tissue
    - M35.9 Systemic involvement of connective tissue, unspecified
  - - M36 Systemic disorders of connective tissue in diseases classified elsewhere
    - M36.0 Dermato(poly)myositis in neoplastic disease
    - M36.1 Arthropathy in neoplastic disease
    - M36.2 Hemophilic arthropathy
    - M36.3 Arthropathy in other blood disorders
    - M36.4 Arthropathy in hypersensitivity reactions classified elsewhere
    - M36.8 Systemic disorders of connective tissue in other diseases classified elsewhere

Instructional Notations

Includes

This note appears immediately under a three character code title to further define, or give examples of, the content of the category.

autoimmune disease NOS
collagen (vascular) disease NOS
systemic autoimmune disease
systemic collagen (vascular) disease

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.

autoimmune disease, single organ or single cell-type -code to relevant condition category

Systemic connective tissue disorders (M30-M36)

Table of Contents

Diseases of the musculoskeletal system and connective tissue (M00–M99)