2022 ICD-10-CM Diagnosis Code M35.0

Sjögren syndrome

    2016 2017 2018 2019 2020 2021 2022 - Revised Code Non-Billable/Non-Specific Code
  • M35.0 should not be used for reimbursement purposes as there are multiple codes below it that contain a greater level of detail.
  • Short description: Sjogren syndrome
  • ICD-10-CM M35.0 is a revised 2022 ICD-10-CM code that became effective on October 1, 2021.
  • This is the American ICD-10-CM version of M35.0 - other international versions of ICD-10 M35.0 may differ.
Applicable To
  • Sicca syndrome
Use Additional
Use Additional Help
Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. For such conditions the ICD-10-CM has a coding convention that requires the underlying condition be sequenced first followed by the manifestation. Wherever such a combination exists there is a "use additional code" note at the etiology code, and a "code first" note at the manifestation code. These instructional notes indicate the proper sequencing order of the codes, etiology followed by manifestation. In most cases the manifestation codes will have in the code title, "in diseases classified elsewhere." Codes with this title are a component of the etiology/manifestation convention. The code title indicates that it is a manifestation code. "In diseases classified elsewhere" codes are never permitted to be used as first listed or principle diagnosis codes. They must be used in conjunction with an underlying condition code and they must be listed following the underlying condition.
  • code to identify associated manifestations
Type 1 Excludes
Type 1 Excludes Help
A type 1 excludes note is a pure excludes. It means "not coded here". A type 1 excludes note indicates that the code excluded should never be used at the same time as M35.0. A type 1 excludes note is for used for when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
  • dry mouth, unspecified (
    ICD-10-CM Diagnosis Code R68.2

    Dry mouth, unspecified

      2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
    Type 1 Excludes
    • dry mouth due to dehydration (E86.0)
    • dry mouth due to Sjögren syndrome (M35.0-)
    • salivary gland hyposecretion (K11.7)
    R68.2
    )
The following code(s) above M35.0 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to M35.0:
  • M00-M99
    2022 ICD-10-CM Range M00-M99

    Diseases of the musculoskeletal system and connective tissue

    Note
    • Use an external cause code following the code for the musculoskeletal condition, if applicable, to identify the cause of the musculoskeletal condition
    Type 2 Excludes
    • arthropathic psoriasis (L40.5-)
    • certain conditions originating in the perinatal period (P04-P96)
    • certain infectious and parasitic diseases (A00-B99)
    • compartment syndrome (traumatic) (T79.A-)
    • complications of pregnancy, childbirth and the puerperium (O00-O9A)
    • congenital malformations, deformations, and chromosomal abnormalities (Q00-Q99)
    • endocrine, nutritional and metabolic diseases (E00-E88)
    • injury, poisoning and certain other consequences of external causes (S00-T88)
    • neoplasms (C00-D49)
    • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
    Diseases of the musculoskeletal system and connective tissue
  • M30-M36
    2022 ICD-10-CM Range M30-M36

    Systemic connective tissue disorders

    Includes
    • autoimmune disease NOS
    • collagen (vascular) disease NOS
    • systemic autoimmune disease
    • systemic collagen (vascular) disease
    Type 1 Excludes
    • autoimmune disease, single organ or single cell-type -code to relevant condition category
    Systemic connective tissue disorders
  • M35
    ICD-10-CM Diagnosis Code M35

    Other systemic involvement of connective tissue

      2016 2017 2018 2019 2020 2021 2022 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • reactive perforating collagenosis (L87.1)
    Other systemic involvement of connective tissue
Clinical Information
  • A chronic inflammation of the tear and salivary glands, often accompanied by rheumatoid arthritis and the presence of autoantibodies in the blood
  • An autoimmune disease that affects the tear glands and salivary glands, and may affect glands in the stomach, pancreas, and intestines. The disease causes dry eyes and mouth, and may cause dryness in the nose, throat, air passages, skin, and vagina. It may also cause inflammation in the joints, muscles, and skin; pneumonia; tingling in the fingers and toes; and fatigue. It often occurs with rheumatoid arthritis or other connective tissue diseases.
  • An autoimmune disorder affecting the salivary and lacrimal glands. Morphologically, it is characterized by the presence of lymphocytic and plasmacytic infiltrates which cause destruction of these glands. It results in dry mouth and dry eyes. It may be associated with the presence of other autoimmune disorders, including rheumatoid arthritis and lupus erythematosus.
  • Chronic inflammatory and autoimmune disease in which the salivary and lacrimal glands undergo progressive destruction by lymphocytes and plasma cells resulting in decreased production of saliva and tears. The primary form, often called sicca syndrome, involves both keratoconjunctivitis sicca and xerostomia. The secondary form includes, in addition, the presence of a connective tissue disease, usually rheumatoid arthritis.
  • Sjogren's syndrome is a disease that causes dryness in your mouth and eyes. It can also lead to dryness in other places that need moisture, such as your nose, throat and skin. Most people who get sjogren's syndrome are older than 40. Nine of 10 are women. Sjogren's syndrome is sometimes linked to rheumatic problems such as rheumatoid arthritis.sjogren's syndrome is an autoimmune disease. If you have an autoimmune disease, your immune system, which is supposed to fight disease, mistakenly attacks parts of your own body. In sjogren's syndrome, your immune system attacks the glands that make tears and saliva. It may also affect your joints, lungs, kidneys, blood vessels, digestive organs and nerves. The main symptoms are:
    • dry eyes
    • dry mouth
    treatment focuses on relieving symptoms. nih: national institute of arthritis and musculoskeletal and skin diseases
  • Symptom complex of unknown etiology, usually occurring in middle-aged or older women, marked by the triad of keratoconjunctivitis sicca with or without lacrimal gland enlargement, xerostomia with or without salivary gland enlargement, and the presence of a connective tissue disease, usually rheumatoid arthritis but sometimes systemic lupus erythematosus, scleroderma, or polymyositis; an abnormal immune response has been implicated.
Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change
  • 2021 (effective 10/1/2020): No change
  • 2022 (effective 10/1/2021): Revised code
    • New description: Sjogren syndrome
    • 2021 description: Sicca syndrome [Sjogren]
Code annotations containing back-references to M35.0:
  • Code Also: I27.21
    ICD-10-CM Diagnosis Code I27.21

    Secondary pulmonary arterial hypertension

      2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
    Applicable To
    • (Associated) (drug-induced) (toxin-induced) pulmonary arterial hypertension NOS
    • (Associated) (drug-induced) (toxin-induced) (secondary) group 1 pulmonary hypertension
    Code Also
    • associated conditions if applicable, or adverse effects of drugs or toxins, such as:
    • adverse effect of appetite depressants (T50.5X5)
    • congenital heart disease (Q20-Q28)
    • human immunodeficiency virus [HIV] disease (B20)
    • polymyositis (M33.2-)
    • portal hypertension (K76.6)
    • rheumatoid arthritis (M05.-)
    • schistosomiasis (B65.-)
    • Sjögren syndrome (M35.0-)
    • systemic sclerosis (M34.-)
  • Type 1 Excludes: K11.8
    , R68.2
    ICD-10-CM Diagnosis Code K11.8

    Other diseases of salivary glands

      2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
    Applicable To
    • Benign lymphoepithelial lesion of salivary gland
    • Mikulicz' disease
    • Necrotizing sialometaplasia
    • Sialectasia
    • Stenosis of salivary duct
    • Stricture of salivary duct
    Type 1 Excludes
    ICD-10-CM Diagnosis Code R68.2

    Dry mouth, unspecified

      2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
    Type 1 Excludes
    • dry mouth due to dehydration (E86.0)
    • dry mouth due to Sjögren syndrome (M35.0-)
    • salivary gland hyposecretion (K11.7)

Diagnosis Index entries containing back-references to M35.0:
  • Keratoconjunctivitis H16.20-
    ICD-10-CM Diagnosis Code H16.20-

    Unspecified keratoconjunctivitis

      2016 2017 2018 2019 2020 2021 2022 Non-Billable/Non-Specific Code
    Applicable To
    • Superficial keratitis with conjunctivitis NOS
    • sicca (Sjogren's) M35.0-

ICD-10-CM Codes Adjacent To M35.0
M34.0 Progressive systemic sclerosis
M34.1 CR(E)ST syndrome
M34.2 Systemic sclerosis induced by drug and chemical
M34.8 Other forms of systemic sclerosis
M34.81 Systemic sclerosis with lung involvement
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
M35.0 Sjögren syndrome
M35.00 …… unspecified
M35.01 …… with keratoconjunctivitis
M35.02 …… with lung involvement
M35.03 …… with myopathy
M35.04 …… with tubulo-interstitial nephropathy
M35.05 …… with inflammatory arthritis
M35.06 …… with peripheral nervous system involvement
M35.07 …… with central nervous system involvement
M35.08 …… with gastrointestinal involvement
M35.0A …… with glomerular disease

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.