International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10)-WHO Version for ;2019-covid-expanded

Chapter XIII
Diseases of the musculoskeletal system and connective tissue
(M00-M99)

Systemic connective tissue disorders
(M30-M36)

Incl.:
autoimmune disease:
  • NOS
  • systemic
collagen (vascular) disease:
  • NOS
  • systemic
Excl.:
antiphospholipid syndrome (D68.6)
autoimmune disease, single organ or single cell-type (code to relevant condition category)

M30 Polyarteritis nodosa and related conditions

M30.0 Polyarteritis nodosa
M30.1 Polyarteritis with lung involvement [Churg-Strauss]
Incl.:
Allergic granulomatous angiitis
M30.2 Juvenile polyarteritis
M30.3 Mucocutaneous lymph node syndrome [Kawasaki]
M30.8 Other conditions related to polyarteritis nodosa
Incl.:
Polyangiitis overlap syndrome

M31 Other necrotizing vasculopathies

M31.0 Hypersensitivity angiitis
Incl.:
Goodpasture syndrome
M31.1 Thrombotic microangiopathy
Incl.:
Thrombotic thrombocytopenic purpura
M31.3 Wegener granulomatosis
Incl.:
Granulomatosis with polyangiitis with:
  • kidney involvement† (N08.5*)
  • lung involvement† (J99.1*)
Necrotizing respiratory granulomatosis
M31.4 Aortic arch syndrome [Takayasu]
M31.5 Giant cell arteritis with polymyalgia rheumatica
M31.6 Other giant cell arteritis
M31.7 Microscopic polyangiitis
Incl.:
Microscopic polyarteritis
Excl.:
polyarteritis nodosa (M30.0)
M31.8 Other specified necrotizing vasculopathies
Incl.:
Hypocomplementaemic vasculitis
M31.9 Necrotizing vasculopathy, unspecified

M32 Systemic lupus erythematosus

Excl.:
lupus erythematosus (discoid)(NOS) (L93.0)
M32.0 Drug-induced systemic lupus erythematosus
Coding-Hint
Use additional external cause code (Chapter XX), if desired, to identify drug.
M32.1† Systemic lupus erythematosus with organ or system involvement
Incl.:
Libman-Sacks disease (I39.-*)
Lupus pericarditis (I32.8*)
Systemic lupus erythematosus with:
M32.8 Other forms of systemic lupus erythematosus
M32.9 Systemic lupus erythematosus, unspecified

M33 Dermatopolymyositis

M33.0 Juvenile dermatomyositis
M33.1 Other dermatomyositis
M33.2 Polymyositis
M33.9 Dermatopolymyositis, unspecified

M34 Systemic sclerosis

Incl.:
scleroderma
Excl.:
scleroderma:
M34.0 Progressive systemic sclerosis
M34.1 CR(E)ST syndrome
Additional Text

Combination of calcinosis, Raynaud phenomenon, (o)esophageal dysfunction, sclerodactyly, telangiectasia.

M34.2 Systemic sclerosis induced by drugs and chemicals
Coding-Hint
Use additional external cause code (Chapter XX), if desired, to identify cause.
M34.8 Other forms of systemic sclerosis
Incl.:
Systemic sclerosis with:
M34.9 Systemic sclerosis, unspecified

M35 Other systemic involvement of connective tissue

Excl.:
reactive perforating collagenosis (L87.1)
M35.0 Sicca syndrome [Sjögren]
Incl.:
Sjögren syndrome with:
  • keratoconjunctivitis† (H19.3*)
  • lung involvement† (J99.1*)
  • myopathy† (G73.7*)
  • renal tubulo-interstitial disorders† (N16.4*)
M35.1 Other overlap syndromes
Incl.:
Mixed connective tissue disease
Excl.:
polyangiitis overlap syndrome (M30.8)
M35.2 Behçet disease
M35.3 Polymyalgia rheumatica
Excl.:
polymyalgia rheumatica with giant cell arteritis (M31.5)
M35.4 Diffuse (eosinophilic) fasciitis
M35.5 Multifocal fibrosclerosis
M35.6 Relapsing panniculitis [Weber-Christian]
Excl.:
panniculitis:
M35.7 Hypermobility syndrome
Incl.:
Familial ligamentous laxity
Excl.:
Ehlers-Danlos syndrome (Q79.6)
ligamentous laxity NOS (M24.2)
M35.8 Other specified systemic involvement of connective tissue
M35.9 Systemic involvement of connective tissue, unspecified
Incl.:
Autoimmune disease (systemic) NOS
Collagen (vascular) disease NOS

M36* Systemic disorders of connective tissue in diseases classified elsewhere

Excl.:
arthropathies in diseases classified elsewhere (M14.-*)
M36.0* Dermato(poly)myositis in neoplastic disease (C00-D48†)
M36.1* Arthropathy in neoplastic disease (C00-D48†)
Incl.:
Arthropathy in:
M36.2* Haemophilic arthropathy (D66-D68†)
M36.3* Arthropathy in other blood disorders (D50-D76†)
Excl.:
arthropathy in Henoch(-Schönlein) purpura (M36.4*)
M36.4* Arthropathy in hypersensitivity reactions classified elsewhere
Incl.:
Arthropathy in Henoch(-Schönlein) purpura (D69.0†)
M36.8* Systemic disorders of connective tissue in other diseases classified elsewhere
Incl.:
Systemic disorders of connective tissue in: