ICD-10-CM Book 2024 Edition

Chapters  /  Chapter 13  /  M30-M36

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
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