Chapter XIII

Diseases of the musculoskeletal system and connective tissue

Systemic connective tissue disorders

Includes: autoimmune disease:
· systemic
collagen (vascular) disease:
· systemic
Excludes: 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]
Allergic granulomatous angiitis
M30.2 Juvenile polyarteritis
M30.3 Mucocutaneous lymph node syndrome [Kawasaki]
M30.8 Other conditions related to polyarteritis nodosa
Polyangiitis overlap syndrome

M31 Other necrotizing vasculopathies
M31.0 Hypersensitivity angiitis
Goodpasture's syndrome
M31.1 Thrombotic microangiopathy
Thrombotic thrombocytopenic purpura
M31.2 Lethal midline granuloma
M31.3 Wegener's granulomatosis
Necrotizing respiratory granulomatosis
M31.4 Aortic arch syndrome [Takayasu]
M31.5 Giant cell arteritis with polymyalgia rheumatica
Other giant cell arteritis
Temporal arteritis
M31.7 Microscopic polyangiitis
Microscopic polyarteritis
Excludes: polyarteritis nodosa ( M30.0 )
M31.8 Other specified necrotizing vasculopathies
Hypocomplementaemic vasculitis
M31.9 Necrotizing vasculopathy, unspecified

M32 Systemic lupus erythematosus
Excludes: lupus erythematosus (discoid)(NOS) ( L93.0 )
M32.0 Drug-induced systemic lupus erythematosus
Use additional external cause code (Chapter XX), if desired, to identify drug.
M32.1+ Systemic lupus erythematosus with organ or system involvement
Libman-Sacks disease ( I39.-* )
Lupus pericarditis ( I32.8* )
Systemic lupus erythematosus with:
· kidney involvement ( N08.5* , N16.4* )
· lung involvement ( J99.1* )
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
Includes: scleroderma
Excludes: scleroderma:
· circumscribed ( L94.0 )
· neonatal ( P83.8 )
M34.0 Progressive systemic sclerosis
M34.1 CR(E)ST syndrome
Combination of calcinosis, Raynaud's phenomenon, (o)esophageal dysfunction, sclerodactyly, telangiectasia.
M34.2 Systemic sclerosis induced by drugs and chemicals
Use additional external cause code (Chapter XX), if desired, to identify cause.
M34.8 Other forms of systemic sclerosis
Systemic sclerosis with:
· lung involvement+ ( J99.1* )
· myopathy+ ( G73.7* )
M34.9 Systemic sclerosis, unspecified

M35 Other systemic involvement of connective tissue
Excludes: reactive perforating collagenosis ( L87.1 )
M35.0 Sicca syndrome [Sjögren]
Sjögren's syndrome with:
· keratoconjunctivitis+ ( H19.3* )
· lung involvement+ ( J99.1* )
· myopathy+ ( G73.7* )
· renal tubulo-interstitial disorders+ ( N16.4* )
M35.1 Other overlap syndromes
Mixed connective tissue disease
Excludes: polyangiitis overlap syndrome ( M30.8 )
M35.2 Behçet's disease
M35.3 Polymyalgia rheumatica
Excludes: polymyalgia rheumatica with giant cell arteritis ( M31.5 )
M35.4 Diffuse (eosinophilic) fasciitis
M35.5 Multifocal fibrosclerosis
M35.6 Relapsing panniculitis [Weber-Christian]
Excludes: panniculitis:
· NOS ( M79.3 )
· lupus ( L93.2 )
M35.7 Hypermobility syndrome
Familial ligamentous laxity
Excludes: 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
Autoimmune disease (systemic) NOS
Collagen (vascular) disease NOS

M36* Systemic disorders of connective tissue in diseases classified elsewhere
Excludes: arthropathies in diseases classified elsewhere ( M14.-* )
M36.0* Dermato(poly)myositis in neoplastic disease ( C00-D48+ )
M36.1* Arthropathy in neoplastic disease ( C00-D48+ )
Arthropathy in:
· leukaemia ( C91-C95+ )
· malignant histiocytosis ( C96.1+ )
· multiple myeloma ( C90.0+ )
M36.2* Haemophilic arthropathy ( D66-D68+ )
M36.3* Arthropathy in other blood disorders ( D50-D76+ )
Excludes: arthropathy in Henoch(-Schönlein) purpura ( M36.4* )
M36.4* Arthropathy in hypersensitivity reactions classified elsewhere
Arthropathy in Henoch(-Schönlein) purpura ( D69.0+ )
M36.8* Systemic disorders of connective tissue in other diseases classified elsewhere
Systemic disorders of connective tissue in:
· hypogammaglobulinaemia ( D80.-+ )
· ochronosis ( E70.2+ )