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

M31.2 Lethal midline granuloma

M31.3 Wegener granulomatosis

Incl.:

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:

• 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

Incl.:

scleroderma

Excl.:

scleroderma:

• circumscribed (L94.0)
• neonatal (P83.8)

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:

• lung involvement† (J99.1*)
• myopathy† (G73.7*)

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:

• NOS (M79.3)
• lupus (L93.2)

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:

• leukaemia (C91-C95†)
• malignant histiocytosis (C96.8†)
• multiple myeloma (C90.0†)

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:

• hypogammaglobulinaemia (D80.-†)
• ochronosis (E70.2†)