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Systemic involvement of connective tissue, unspecified
disease; Hemolytic anemia with systemic lupus erythematosus; Interstitial lung disease due to collagen vascular disease; Left autoimmune inner ear disease; Left autoimmune sensorineural hearing loss; Left sided autoimmune disorder of inner ear; Lung disease in collagen vascular disease; Mixed collagen vascular disease; Mixed connective tissue disease; Polyneuropathy (multiple nerve disorder...; Both sides autoimmune inner ear disease; Both sides autoimmune sensorineural hearing loss; Collagen disease; Connective tissue disease w systemic involvement; Connective tissue disease, systemic involvement; Connective tissue disorder; Disorder of connective tissue; Disorder of connective tissue with systemic involvement; Hemolytic anemia associated with chronic inflammatory disease; Hemolytic... connective tissue disease; Autoimmune disease (systemic) NOS; Collagen (vascular) disease NOS...Autoimmune disease; Autoimmune disorder; Autoimmune disorder of inner ear; Autoimmune inner ear
ICD-10-CM Diagnosis Code M35.9

Systemic involvement of connective tissue, unspecified

    2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
Applicable To
  • Autoimmune disease (systemic) NOS
  • Collagen (vascular) disease NOS
Family history of other diseases of the musculoskeletal system and connective tissue
Family history of diseases of the ms sys and connective tiss; Family history of connective tissue disorder; Family history of musculoskeletal disease; Family history of systemic lupus erythematosus; Fh: musculoskeletal disease
ICD-10-CM Diagnosis Code Z82.69

Family history of other diseases of the musculoskeletal system and connective tissue

    2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code POA Exempt
Localized connective tissue disorder, unspecified
Dilated cardiomyopathy associated with connective tissue disorder; Dilated cardiomyopathy due to connective tissue di; Lung disease due to connective tissue disorder
ICD-10-CM Diagnosis Code L94.9

Localized connective tissue disorder, unspecified

    2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
Other specified systemic involvement of connective tissue
Connective tissue disease overlap syndrome; Eosinophilia myalgia syndrome; Fasciitis with eosinophilia syndrome
ICD-10-CM Diagnosis Code M35.8

Other specified systemic involvement of connective tissue

    2016 2017 2018 2019 2020 2021 2022 Non-Billable/Non-Specific Code
Other localized connective tissue disorders
systemic connective tissue disorders (M30-M36)
ICD-10-CM Diagnosis Code L94
  • L94 Other localized connective tissue disorders
    • L94.0 Localized scleroderma [morphea]
    • L94.1 Linear scleroderma
    • L94.2 Calcinosis cutis
    • L94.3 Sclerodactyly
    • L94.4 Gottron's papules
    • L94.5 Poikiloderma vasculare atrophicans
    • L94.6 Ainhum
    • L94.8 Other specified localized connective tissue d...
    • L94.9 Localized connective tissue disorder, unspeci...
Other overlap syndromes
Mixed collagen vascular disease; Mixed connective tissue disease; polyangiitis overlap syndrome (M30.8); Mixed connective tissue disease
ICD-10-CM Diagnosis Code M35.1

Other overlap syndromes

    2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
Applicable To
  • Mixed connective tissue disease
Type 1 Excludes
  • polyangiitis overlap syndrome (M30.8)
Personal history of other diseases of the musculoskeletal system and connective tissue
Personal history of diseases of the ms sys and conn tiss; H/o: arthritis; H/o: gout; H/o: musculoskeletal disease; History of acquired spondylolisthesis; History of arthritis; History of disfigurement of limb (arm or leg); History of fibromatosis; History of fibromatosis (multiple benign soft tissue tumors); History of gout; History of kawasaki disease; History of kawasakis syndrome; History of limb disfigurement; History of musculoskeletal disorder; History of osteomyelitis; History of osteomyelitis (bone infection); History of osteopenia; History of polymyalgia rheumatica; History of rheumatoid arthritis in remission; Rheumatoid arthritis in remission
ICD-10-CM Diagnosis Code Z87.39

Personal history of other diseases of the musculoskeletal system and connective tissue

    2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code POA Exempt
Benign neoplasm of connective and other soft tissue, unspecified
Benign neoplasm of connective and other soft tissue, unsp; Benign neoplasm of soft tissue; Fibroma; Leiomyoma; Myxoma
ICD-10-CM Diagnosis Code D21.9

Benign neoplasm of connective and other soft tissue, unspecified

    2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
Benign neoplasm of connective and other soft tissue of head, face and neck
Benign neoplasm of connctv/soft tiss of head, face and neck; Benign neoplasm head, soft tissue; Benign neoplasm neck, soft tissue; Benign neoplasm of soft tissues of face; Benign neoplasm of soft tissues of head; Benign neoplasm of soft tissues of neck; Benign neoplasm, soft tissue of face; Neuroma of face; benign neoplasm of connective tissue of orbit (D31.6-); Benign neoplasm of connective tissue of ear; Benign neoplasm of connective tissue of eyelid
ICD-10-CM Diagnosis Code D21.0

Benign neoplasm of connective and other soft tissue of head, face and neck

    2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
Applicable To
  • Benign neoplasm of connective tissue of ear
  • Benign neoplasm of connective tissue of eyelid
Type 1 Excludes
  • benign neoplasm of connective tissue of orbit (D31.6-)
Benign neoplasm of connective and other soft tissue of thorax
Benign neoplasm of connective and oth soft tissue of thorax; Benign neoplasm of soft tissues of thorax; Benign neoplasm thorax, soft tissue; benign neoplasm of heart (D15.1); benign neoplasm of mediastinum (D15.2); benign neoplasm of thymus (D15.0); Benign neoplasm of axilla; Benign neoplasm of diaphragm; Benign neoplasm of great vessels
ICD-10-CM Diagnosis Code D21.3

Benign neoplasm of connective and other soft tissue of thorax

    2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
Applicable To
  • Benign neoplasm of axilla
  • Benign neoplasm of diaphragm
  • Benign neoplasm of great vessels
Type 1 Excludes
  • benign neoplasm of heart (D15.1)
  • benign neoplasm of mediastinum (D15.2)
  • benign neoplasm of thymus (D15.0)
Benign neoplasm of connective and other soft tissue of pelvis
Benign neoplasm of connective and oth soft tissue of pelvis; Benign neoplasm of muscle of pelvis; Benign neoplasm of soft tissues of pelvis; Benign neoplasm pelvis, muscle; Benign neoplasm soft tissue, pelvis; benign neoplasm of any uterine ligament (D28.2); uterine leiomyoma (D25.-)
ICD-10-CM Diagnosis Code D21.5

Benign neoplasm of connective and other soft tissue of pelvis

    2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
Type 1 Excludes
  • benign neoplasm of any uterine ligament (D28.2)
  • uterine leiomyoma (D25.-)
Benign neoplasm of connective and other soft tissue of trunk, unspecified
Benign neoplasm of connctv/soft tiss of trunk, unsp; Benign neoplasm of soft tissues of trunk; Benign neoplasm trunk, soft tissue; Benign neoplasm of connective and other soft tissue of back NOS
ICD-10-CM Diagnosis Code D21.6

Benign neoplasm of connective and other soft tissue of trunk, unspecified

    2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
Applicable To
  • Benign neoplasm of connective and other soft tissue of back NOS
Systemic disorders of connective tissue in diseases classified elsewhere
Systemic disorders of conn tiss in diseases classd elswhr; arthropathies in diseases classified elsewhere (M14.-)
ICD-10-CM Diagnosis Code M36
  • M36 Systemic disorders of connective tissue in di...
    • M36.0 Dermato(poly)myositis in neoplastic disease
    • M36.1 Arthropathy in neoplastic disease
    • M36.2 Hemophilic arthropathy
    • M36.3 Arthropathy in other blood disorders
    • M36.4 Arthropathy in hypersensitivity reactions cla...
    • M36.8 Systemic disorders of connective tissue in ot...
Neoplasm of uncertain behavior of connective and other soft tissue
of nose (D38.5); neoplasm of uncertain behavior of connective tissue of breast (D48.6-); Neoplasm of uncertain behavior of connective tissue of ear; Neoplasm of uncertain behavior of connective tissue... of tendon sheath; Hemangiopericytoma; Histiocytoma of soft tissue, fibrous, atypical; Lipoma of soft tissue, atypical; Lymphangiomyomatosis; Lymphangiomyomatosis of connective tissue; Neoplasm of uncertain behavior hand, soft tissue; Neoplasm of uncertain behavior nerve of hand; Neoplasm of uncertain behavior of hand nerve; Neoplasm of uncertain behavior of soft tissue; Neoplasm of uncertain behavior of soft tissue of hand; Neoplasm of uncertain behavior of soft tissues; Neoplasm of uncertain... of skin of scalp; Atypical fibroxanthoma, skin; Atypical lipoma of soft tissue; Cancer uncert behavior... of pelvis; Neoplasm of uncertain behavior pelvis, soft tissue; Neoplasm of uncertain behavior, desmoid...Neoplasm of uncertain behavior of connctv/soft tiss; Atypical fibrous histiocytoma of soft tissue
ICD-10-CM Diagnosis Code D48.1

Neoplasm of uncertain behavior of connective and other soft tissue

    2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
Applicable To
  • Neoplasm of uncertain behavior of connective tissue of ear
  • Neoplasm of uncertain behavior of connective tissue of eyelid
  • Stromal tumors of uncertain behavior of digestive system
Type 1 Excludes
  • neoplasm of uncertain behavior of articular cartilage (D48.0)
  • neoplasm of uncertain behavior of cartilage of larynx (D38.0)
  • neoplasm of uncertain behavior of cartilage of nose (D38.5)
  • neoplasm of uncertain behavior of connective tissue of breast (D48.6-)
Systemic disorders of connective tissue in other diseases classified elsewhere
Systemic disord of conn tiss in oth diseases classd elswhr; underlying disease, such as:; alkaptonuria (E70.2); hypogammaglobulinemia (D80.-); ochronosis (E70.2)
ICD-10-CM Diagnosis Code M36.8

Systemic disorders of connective tissue in other diseases classified elsewhere

    2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code Manifestation Code
Code First
  • underlying disease, such as:
  • alkaptonuria (E70.2)
  • hypogammaglobulinemia (D80.-)
  • ochronosis (E70.2)
Malignant neoplasm of connective and soft tissue, unspecified
Malignant neoplasm of connective and soft tissue, unsp; Angiosarcoma; Atypical fibroxanthoma of skin; Atypical fibroxanthoma, skin; Cancer of the soft tissue; Cancer of the synovium, sarcoma; Cancer, angiosarcoma; Cancer, leiomyosarcoma; Cancer, liposarcoma; Cancer, malignant fibrous histiocytoma; Cancer, myxoid liposarcoma; Cancer, rhabdomyosarcoma; Cancer, sarcoma; Ganglioneuroblastoma; Leiomyosarcoma; Liposarcoma; Malignant fibrous histiocytoma; Malignant neoplasm of soft tissue; Myxoid liposarcoma; Rhabdomyosarcoma; Sarcoma; Sarcoma of soft tissue; Sarcoma, soft tissue; Synovial sarcoma
ICD-10-CM Diagnosis Code C49.9

Malignant neoplasm of connective and soft tissue, unspecified

    2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
Benign neoplasm of connective and other soft tissue of abdomen
Benign neoplasm of connective and oth soft tissue of abdomen; Benign ganglioneuroma of abdomen; Benign neoplasm abdomen, soft tissue; Benign neoplasm of soft tissues of abdomen; Benign neoplasm small intestine, leiomyoma; Benign neoplasm stomach, leiomyoma; Ganglioneuroma abdomen, benign; Leiomyoma of small intestine; Leiomyoma of stomach; Benign stromal tumors of abdomen
ICD-10-CM Diagnosis Code D21.4

Benign neoplasm of connective and other soft tissue of abdomen

    2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
Applicable To
  • Benign stromal tumors of abdomen
Malignant neoplasm of overlapping sites of connective and soft tissue
Malignant neoplasm of ovrlp sites of conn and soft tissue; Angiosarcoma, overlapping sites; Cancer of the soft tissue, overlapping sites; Leiomyosarcoma, overlapping sites; Liposarcoma, overlapping sites; Malignant fibrous histiocytoma, overlapping sites; Overlapping malignant neoplasm of soft tissues; Overlapping primary angiosarcoma; Overlapping primary leiomyosarcoma; Overlapping primary liposarcoma; Overlapping primary malignant fibrous histiocytoma; Overlapping primary rhabdomyosarcoma; Overlapping primary sarcoma; Rhabdomyosarcoma, overlapping sites; Sarcoma, overlapping sites; Primary malignant neoplasm of two or more contiguous sites of connective and soft tissue
ICD-10-CM Diagnosis Code C49.8

Malignant neoplasm of overlapping sites of connective and soft tissue

    2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
Applicable To
  • Primary malignant neoplasm of two or more contiguous sites of connective and soft tissue
Personal history of diseases of the musculoskeletal system and connective tissue
Personal history of diseases of the ms sys and conn tiss; personal history of (healed) traumatic fracture (Z87.81); Conditions classifiable to M00-M99
ICD-10-CM Diagnosis Code Z87.3

Personal history of diseases of the musculoskeletal system and connective tissue

    2016 2017 2018 2019 2020 2021 2022 Non-Billable/Non-Specific Code
Applicable To
Type 2 Excludes
  • personal history of (healed) traumatic fracture (Z87.81)
Interstitial lung disease with progressive fibrotic phenotype in diseases classified elsewhere
Interstit lung dis w progr fibrotic phenotype dis classd e; underlying disease, such as:; lung diseases due to external agents (J60-J70); rheumatoid arthritis (M05.00-M06.9); sarcoidosis (D86); systemic connective tissue disorders (M30-M36); Progressive fibrotic interstitial lung disease
ICD-10-CM Diagnosis Code J84.170

Interstitial lung disease with progressive fibrotic phenotype in diseases classified elsewhere

    2021 - New Code 2022 Billable/Specific Code Manifestation Code
Applicable To
  • Progressive fibrotic interstitial lung disease
Code First
  • underlying disease, such as:
  • lung diseases due to external agents (J60-J70)
  • rheumatoid arthritis (M05.00-M06.9)
  • sarcoidosis (D86)
  • systemic connective tissue disorders (M30-M36)
Other acquired deformities of musculoskeletal system and connective tissue
Oth acquired deformities of ms sys and connective tissue; acquired absence of limbs and organs (Z89-Z90); acquired deformities of limbs (M20-M21); congenital malformations and deformations of the musculoskeletal system (Q65-Q79); deforming dorsopathies (M40-M43); dentofacial anomalies [including malocclusion] (M26.-); postprocedural musculoskeletal disorders (M96.-)
ICD-10-CM Diagnosis Code M95
  • M95 Other acquired deformities of musculoskeletal...
    • M95.0 Acquired deformity of nose
    • M95.1 Cauliflower ear
    • M95.2 Other acquired deformity of head
    • M95.3 Acquired deformity of neck
    • M95.4 Acquired deformity of chest and rib
    • M95.5 Acquired deformity of pelvis
    • M95.8 Other specified acquired deformities of muscu...
    • M95.9 Acquired deformity of musculoskeletal system,...
Mixed cellularity Hodgkin lymphoma, unspecified site
Hodgkin's disease, mixed cellularity (clinical); Hodgkins disease, mixed cellularity
ICD-10-CM Diagnosis Code C81.20

Mixed cellularity Hodgkin lymphoma, unspecified site

    2016 2017 - Revised Code 2018 2019 2020 2021 2022 Billable/Specific Code
Malignant neoplasm of other connective and soft tissue
malignant neoplasm of cartilage (of):; articular (C40-C41); larynx (C32.3); nose (C30.0); malignant neoplasm of connective tissue of breast (C50.-); Kaposi's sarcoma of soft tissue (C46.1); malignant neoplasm of heart (C38.0); malignant neoplasm of peripheral nerves and autonomic nervous system (C47.-); malignant neoplasm of peritoneum (C48.2); malignant neoplasm of retroperitoneum (C48.0); malignant neoplasm of uterine ligament (C57.3); mesothelioma (C45.-); malignant neoplasm of blood vessel; malignant neoplasm of bursa; malignant neoplasm of cartilage; malignant neoplasm of fascia; malignant neoplasm of fat; malignant neoplasm of ligament, except uterine; malignant neoplasm of lymphatic vessel; malignant neoplasm of muscle; malignant neoplasm of synovia; malignant neoplasm of tendon (sheath)
ICD-10-CM Diagnosis Code C49
  • C49 Malignant neoplasm of other connective and so...
    • C49.0 Malignant neoplasm of connective and soft tis...
    • C49.1 Malignant neoplasm of connective and soft tis...
      • C49.10 Malignant neoplasm of connective and soft tis...
      • C49.11 Malignant neoplasm of connective and soft tis...
      • C49.12 Malignant neoplasm of connective and soft tis...
    • C49.2 Malignant neoplasm of connective and soft tis...
      • C49.20 Malignant neoplasm of connective and soft tis...
      • C49.21 Malignant neoplasm of connective and soft tis...
      • C49.22 Malignant neoplasm of connective and soft tis...
    • C49.3 Malignant neoplasm of connective and soft tis...
    • C49.4 Malignant neoplasm of connective and soft tis...
    • C49.5 Malignant neoplasm of connective and soft tis...
    • C49.6 Malignant neoplasm of connective and soft tis...
    • C49.8 Malignant neoplasm of overlapping sites of co...
    • C49.9 Malignant neoplasm of connective and soft tis...
    • C49.A Gastrointestinal stromal tumor
      • C49.A0 Gastrointestinal stromal tumor, unspecified s...
      • C49.A1 …… of esophagus
      • C49.A2 …… of stomach
      • C49.A3 Gastrointestinal stromal tumor of small intes...
      • C49.A4 Gastrointestinal stromal tumor of large intes...
      • C49.A5 …… of rectum
      • C49.A9 Gastrointestinal stromal tumor of other sites...
Mixed cellularity Hodgkin lymphoma, spleen
Hodgkin's disease, mixed cellularity of spleen; Lymphoma spleen, hodgkins mixed cellularity
ICD-10-CM Diagnosis Code C81.27

Mixed cellularity Hodgkin lymphoma, spleen

    2016 2017 - Revised Code 2018 2019 2020 2021 2022 Billable/Specific Code
Other benign neoplasms of connective and other soft tissue
benign neoplasm of articular cartilage (D16.-); benign neoplasm of cartilage of larynx (D14.1); benign neoplasm of cartilage of nose (D14.0); benign neoplasm of connective tissue of breast (D24.-); benign neoplasm of peripheral nerves and autonomic nervous system (D36.1-); benign neoplasm of peritoneum (D20.1); benign neoplasm of retroperitoneum (D20.0); benign neoplasm of uterine ligament, any (D28.2); benign neoplasm of vascular tissue (D18.-); hemangioma (D18.0-); lipomatous neoplasm (D17.-); lymphangioma (D18.1); uterine leiomyoma (D25.-); benign neoplasm of blood vessel; benign neoplasm of bursa; benign neoplasm of cartilage; benign neoplasm of fascia; benign neoplasm of fat; benign neoplasm of ligament, except uterine; benign neoplasm of lymphatic channel; benign neoplasm of muscle; benign neoplasm of synovia; benign neoplasm of tendon (sheath); benign stromal tumors
ICD-10-CM Diagnosis Code D21
  • D21 Other benign neoplasms of connective and othe...
    • D21.0 Benign neoplasm of connective and other soft ...
    • D21.1 Benign neoplasm of connective and other soft ...
      • D21.10 Benign neoplasm of connective and other soft ...
      • D21.11 Benign neoplasm of connective and other soft ...
      • D21.12 Benign neoplasm of connective and other soft ...
    • D21.2 Benign neoplasm of connective and other soft ...
      • D21.20 Benign neoplasm of connective and other soft ...
      • D21.21 Benign neoplasm of connective and other soft ...
      • D21.22 Benign neoplasm of connective and other soft ...
    • D21.3 Benign neoplasm of connective and other soft ...
    • D21.4 Benign neoplasm of connective and other soft ...
    • D21.5 Benign neoplasm of connective and other soft ...
    • D21.6 Benign neoplasm of connective and other soft ...
    • D21.9 Benign neoplasm of connective and other soft ...