A00-B99 C00-D48D50-D89 E00-E90 F00-F99 G00-G99 H00-H59 H60-H95 I00-I99 J00-J99 K00-K93
L00-L99 M00-M99 N00-N99 O00-O99 P00-P96 Q00-Q99 R00-R99 S00-T98 V01-Y98 Z00-Z99 U00-U99

M30-M36 Systemic connective tissue disorders

Chapter XIII

Diseases of the musculoskeletal system and connective tissue

Systemic connective tissue disorders

autoimmune disease:
· systemic
collagen (vascular) disease:
· systemic

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

M30Polyarteritis nodosa and related conditions
Latin:Polyarteritis nodosa et morbi affines
M30.0Polyarteritis nodosa
Latin: Polyarteritis nodosa
M30.1Polyarteritis with lung involvement [Churg-Strauss]
Latin: Polyarteritis pulmonis (Churg-Strauss)
Allergic granulomatous angiitis
M30.2Juvenile polyarteritis
Latin: Polyarteritis juvenilis
M30.3 Mucocutaneous lymph node syndrome [Kawasaki]
Latin: Syndroma nodi lymphatici mucocutanea (Kawasaki)
M30.8 Other conditions related to polyarteritis nodosa
Latin: Status similes cum polyarteritide nodosa connexi alii
Polyangiitis overlap syndrome
M31Other necrotizing vasculopathies
Latin: Vasculopathiae necroticae aliae
M31.0Hypersensitivity angiitis
Latin: Angiitis hypersensitiva
Goodpasture's syndrome
M31.1 Thrombotic microangiopathy
Latin: Microangiopathia thrombotica
Thrombotic thrombocytopenic purpura
M31.2 Lethal midline granuloma
Latin: Granuloma centrale letale
M31.3 Wegener's granulomatosis
Latin: Granulomatosis Wegner
Necrotizing respiratory granulomatosis
M31.4 Aortic arch syndrome [Takayasu]
Latin: Syndroma arcus aortae (Takayasu)
M31.5 Giant cell arteritis with polymyalgia rheumatica
Latin: Arteritis gigantocellularis cum polymyalgia rheumatica
M31.6 Other giant cell arteritis
Latin: Arteritis gigantocellularis alia
M31.7Microscopic polyangiitis
Latin: .
Microscopic polyarteritis
Excludes:polyarteritis nodosa (M30.0)
M31.8Other specified necrotizing vasculopathies
Latin: Vasculopathiae necroticae aliae, specificatae
Hypocomplementaemic vasculitis
M31.9 Necrotizing vasculopathy, unspecified
Latin: Vasculopathia necrotica, non specificata
M32Systemic lupus erythematosus
Latin: Lupus erythematosus systemicus
Excludes: lupus erythematosus (discoid)(NOS) (L93.0)
M32.0Drug-induced systemic lupus erythematosus
Latin: Lupus erythematosus systematicus medicamentosus
Use additional external cause code (Chapter XX), if desired, to identify drug.
M32.1+Systemic lupus erythematosus with organ or system involvement
Latin: Lupus erythematosus systemicus cum organo sive systemate apprehendenti
Libman-Sacks disease (I39.-*)
Lupus pericarditis (I32.8*)
Systemic lupus erythematosus with:
· kidney involvement (N08.5*, N16.4*)
· lung involvement (J99.1*)
M32.8Other forms of systemic lupus erythematosus
Latin: Lupus erythematosus systemicus, formae aliae
M32.9Systemic lupus erythematosus, unspecified
Latin: Lupus erythematosus systemicus, non specificatus
Latin: Dermatopolymyositis
M33.0 Juvenile dermatomyositis
Latin: Dermatomyositis puerilis
M33.1 Other dermatomyositis
Latin: Dermatomyositis alia
Latin: Polymyositis
M33.9Dermatopolymyositis, unspecified
Latin: Dermatopolymyositis, non specificata
M34Systemic sclerosis
Latin: Scleroderma
· circumscribed (L94.0)
· neonatal (P83.8)
M34.0Progressive systemic sclerosis
Latin: Scleroderma progressivum diffusum
M34.1CR(E)ST syndrome
Latin: Syndroma CREST
Combination of calcinosis, Raynaud's phenomenon, (o)esophageal dysfunction, sclerodactyly, telangiectasia.
M34.2Systemic sclerosis induced by drugs and chemicals
Latin: Scleroderma medicamentosum et chemicalis
Use additional external cause code (Chapter XX), if desired, to identify cause.
M34.8Other forms of systemic sclerosis
Latin: Scleroderma, formae aliae
Systemic sclerosis with:
· lung involvement+ (J99.1*)
· myopathy+ (G73.7*)
M34.9Systemic sclerosis, unspecified
Latin: Scleroderma, non specificatum
M35Other systemic involvement of connective tissue
Latin: Morbi textus connexivi systemici alii
Excludes:reactive perforating collagenosis (L87.1)
M35.0Sicca syndrome [Sjögren]
Latin: Syndroma sicca (Sjogren)
Sjögren's syndrome with:
· keratoconjunctivitis+ (H19.3*)
· lung involvement+ (J99.1*)
· myopathy+ (G73.7*)
· renal tubulo-interstitial disorders+ (N16.4*)
M35.1Other overlap syndromes
Latin: Syndromae flectentes aliae
Mixed connective tissue disease
Excludes:polyangiitis overlap syndrome (M30.8)
M35.2Behçet's disease
Latin: Morbus Behcet
M35.3Polymyalgia rheumatica
Latin: Polymyalgia rheumatica
Excludes: polymyalgia rheumatica with giant cell arteritis (M31.5)
M35.4 Diffuse (eosinophilic) fasciitis
Latin: Fasciitis diffusa (eosinophilica)
M35.5Multifocal fibrosclerosis
Latin: Fibrosclerosis multifocalis
M35.6Relapsing panniculitis [Weber-Christian]
Latin: Panniculitis recidiva (Weber-Christian)
· NOS (M79.3)
· lupus (L93.2)
M35.7 Hypermobility syndrome
Latin: Syndroma hypermobilitatis
Familial ligamentous laxity
Excludes:Ehlers-Danlos syndrome (Q79.6)
ligamentous laxity NOS (M24.2)
M35.8Other specified systemic involvement of connective tissue
Latin: Morbi textus connexivi systemici alii, specificati
M35.9Systemic involvement of connective tissue, unspecified
Latin: Morbus textus connexivi systemicus alius, non specificatus
Autoimmune disease (systemic) NOS
Collagen (vascular) disease NOS
M36*Systemic disorders of connective tissue in diseases classified elsewhere
Latin: Morbi textus connexivi systemici in morbis aliis
Excludes: arthropathies in diseases classified elsewhere (M14.-*)
M36.0*Dermato(poly)myositis in neoplastic disease (C00-D48+)
Latin: Dermato (poly) myositis neoplasmosa (C00-D48+)
M36.1* Arthropathy in neoplastic disease (C00-D48+)
Latin: Arthropathia neoplasmosa (C00-D48+)
Arthropathy in:
· leukaemia (C91-C95+)
· malignant histiocytosis (C96.1+)
· multiple myeloma (C90.0+)
M36.2* Haemophilic arthropathy (D66-D68+)
Latin: Arthropathia haemophilica (D66-D68+)
M36.3*Arthropathy in other blood disorders (D50-D76+)
Latin: Arthropathia in morbis sanguinis aliis (D50-D76+)
Excludes:arthropathy in Henoch(-Schönlein) purpura (M36.4*)
M36.4*Arthropathy in hypersensitivity reactions classified elsewhere
Latin: Arthropathia allergica, alia
Arthropathy in Henoch(-Schönlein) purpura (D69.0+)
M36.8*Systemic disorders of connective tissue in other diseases classified elsewhere
Latin: Morbi textus connexivi systemici in morbis aliis
Systemic disorders of connective tissue in:
· hypogammaglobulinaemia (D80.-+)
· ochronosis (E70.2+)