Coding: ICD-10-CM Section M30-M36

ICD-10-CM Book 2026 Edition

Systemic connective tissue disorders (M30-M36)

Includes:	autoimmune disease NOS collagen (vascular) disease NOS systemic autoimmune disease systemic collagen (vascular) disease
Excludes1:	autoimmune disease, single organ or single cell-type -code to relevant condition category

M30

Polyarteritis nodosa and related conditions

Excludes1

microscopic polyarteritis (M31.7)

M30.0

Polyarteritis nodosa

M30.1

Polyarteritis with lung involvement [Churg-Strauss]

Allergic granulomatous angiitis

Eosinophilic granulomatosis with polyangiitis [EGPA]

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

M31.10

Thrombotic microangiopathy, unspecified

M31.11

Hematopoietic stem cell transplantation-associated thrombotic microangiopathy [HSCT-TMA]

Transplant-associated thrombotic microangiopathy [TA-TMA]
CodeFirst	if applicable: complications of bone marrow transplant (T86.0-) complications of stem cell transplant (T86.5)
UseAdditionalCode	code to identify specific organ dysfunction, such as: acute kidney failure (N17.-) acute respiratory distress syndrome (J80) capillary leak syndrome (I78.8) diffuse alveolar hemorrhage (R04.89) encephalopathy (metabolic) (septic) (G93.41) fluid overload, unspecified (E87.70) graft versus host disease (D89.81-) hemolytic uremic syndrome (D59.3-) hepatic failure (K72.-) hepatic veno-occlusive disease (K76.5) idiopathic interstitial pneumonia (J84.11-) sinusoidal obstruction syndrome (K76.5)

M31.19

Other thrombotic microangiopathy

Thrombotic thrombocytopenic purpura

M31.2

Lethal midline granuloma

M31.3

Wegener's granulomatosis

Granulomatosis with polyangiitis

Necrotizing respiratory granulomatosis

M31.30

Wegener's granulomatosis without renal involvement

Wegener's granulomatosis NOS

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

Microscopic polyarteritis
Excludes1	polyarteritis nodosa (M30.0)

M31.8

Other specified necrotizing vasculopathies

Hypocomplementemic vasculitis

Septic vasculitis

M31.9

Necrotizing vasculopathy, unspecified

M32

Systemic lupus erythematosus (SLE)

Excludes1

lupus erythematosus (discoid) (NOS) (L93.0)

M32.0

Drug-induced systemic lupus erythematosus

UseAdditionalCode

code for adverse effect, if applicable, to identify drug (T36-T50 with fifth or sixth character 5)

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

Libman-Sacks disease

M32.12

Pericarditis in systemic lupus erythematosus

Lupus pericarditis

M32.13

Lung involvement in systemic lupus erythematosus

Pleural effusion due to systemic lupus erythematosus

M32.14

Glomerular disease in systemic lupus erythematosus

Lupus renal disease NOS

M32.15

Tubulo-interstitial nephropathy in systemic lupus erythematosus

M32.19

Other organ or system involvement in systemic lupus erythematosus

UseAdditionalCode

code(s) to identify organ or system involvement, such as encephalitis (G05.3)

M32.8

Other forms of systemic lupus erythematosus

M32.9

Systemic lupus erythematosus, unspecified

SLE NOS

Systemic lupus erythematosus NOS

Systemic lupus erythematosus without organ involvement

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

Adult 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

Dermatomyositis NOS

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]

Excludes1

circumscribed scleroderma (L94.0)
neonatal scleroderma (P83.88)

M34.0

Progressive systemic sclerosis

M34.1

CR(E)ST syndrome

Combination of calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, telangiectasia

M34.2

Systemic sclerosis induced by drug and chemical

CodeFirst	poisoning due to drug or toxin, if applicable (T36-T65 with fifth or sixth character 1-4)
UseAdditionalCode	code for adverse effect, if applicable, to identify drug (T36-T50 with fifth or sixth character 5)

M34.8

Other forms of systemic sclerosis

M34.81

Systemic sclerosis with lung involvement

CodeAlso

if applicable:
other interstitial pulmonary diseases (J84.89)
secondary pulmonary arterial hypertension (I27.21)

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

Excludes1

reactive perforating collagenosis (L87.1)

M35.0

Sjögren syndrome

Sicca syndrome
UseAdditionalCode	code to identify associated manifestations
Excludes1	dry mouth, unspecified (R68.2)

M35.00

Sjögren syndrome, unspecified

M35.01

Sjögren syndrome with keratoconjunctivitis

M35.02

Sjögren syndrome with lung involvement

M35.03

Sjögren syndrome with myopathy

M35.04

Sjögren syndrome with tubulo-interstitial nephropathy

Renal tubular acidosis in sicca syndrome

M35.05

Sjögren syndrome with inflammatory arthritis

M35.06

Sjögren syndrome with peripheral nervous system involvement

M35.07

Sjögren syndrome with central nervous system involvement

M35.08

Sjögren syndrome with gastrointestinal involvement

M35.0A

Sjögren syndrome with glomerular disease

M35.0B

Sjögren syndrome with vasculitis

M35.0C

Sjögren syndrome with dental involvement

M35.09

Sjögren syndrome with other organ involvement

M35.1

Other overlap syndromes

Mixed connective tissue disease
Excludes1	polyangiitis overlap syndrome (M30.8)

M35.2

Behçet's disease

M35.3

Polymyalgia rheumatica

Excludes1

polymyalgia rheumatica with giant cell arteritis (M31.5)

M35.4

Diffuse (eosinophilic) fasciitis

M35.5

Multifocal fibrosclerosis

M35.6

Relapsing panniculitis [Weber-Christian]

Excludes1

lupus panniculitis (L93.2)
panniculitis NOS (M79.3-)

M35.7

Hypermobility syndrome

Familial ligamentous laxity
Excludes1	ligamentous laxity, NOS (M24.2-)
Excludes2	Ehlers-Danlos syndromes (Q79.6-)

M35.8

Other specified systemic involvement of connective tissue

M35.81

Multisystem inflammatory syndrome

MIS-A
MIS-C
Multisystem inflammatory syndrome in adults
Multisystem inflammatory syndrome in children
Pediatric inflammatory multisystem syndrome
PIMS
CodeFirst	, if applicable, COVID-19 (U07.1)
CodeAlso	any associated complications such as: acute hepatic failure (K72.0-) acute kidney failure (N17.-) acute myocarditis (I40.-) acute respiratory distress syndrome (J80) cardiac arrhythmia (I47-I49.-) pneumonia due to COVID-19 (J12.82) severe sepsis (R65.2-) viral cardiomyopathy (B33.24) viral pericarditis (B33.23)
UseAdditionalCode	code, if applicable, for: exposure to COVID-19 or SARS-CoV-2 infection (Z20.822) personal history of COVID-19 (Z86.16) post COVID-19 condition (U09.9)

M35.89

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

Excludes2

arthropathies in diseases classified elsewhere (M14.-)

M36.0

Dermato(poly)myositis in neoplastic disease

CodeFirst	underlying neoplasm (C00-D49)
nonPrimary	Yes

M36.1

Arthropathy in neoplastic disease

CodeFirst	underlying neoplasm, such as: leukemia (C91-C95) malignant histiocytosis (C96.A) multiple myeloma (C90.0)
nonPrimary	Yes

M36.2

Hemophilic arthropathy

Hemarthrosis in hemophilic arthropathy
CodeFirst	underlying disease, such as: factor VIII deficiency (D66) with vascular defect (D68.0-) factor IX deficiency (D67) hemophilia (classical) (D66) hemophilia B (D67) hemophilia C (D68.1)
nonPrimary	Yes

M36.3

Arthropathy in other blood disorders

CodeFirst	underlying disease, such as: disease of blood and blood-forming organs, unspecified (D75.9) other hemoglobinopathies (D58.2) thalassemia (D56.-)
nonPrimary	Yes

M36.4

Arthropathy in hypersensitivity reactions classified elsewhere

CodeFirst	underlying disease, such as: Henoch (-Schönlein) purpura (D69.0) serum sickness (T80.6-)
nonPrimary	Yes

M36.8

Systemic disorders of connective tissue in other diseases classified elsewhere

CodeFirst	underlying disease, such as: alkaptonuria (E70.29) hypogammaglobulinemia (D80.-) ochronosis (E70.29)
nonPrimary	Yes