M35.03 ICD-10-CM Code: Sjögren syndrome with myopathy
Compare with another codeCheck this code on a claim
Billing Status: YES. This is a valid, specific, and billable ICD-10-CM reference.
Coding at a Glance
- Tabular directives
- 4 inclusion terms · 3 Excludes1 · 1 use-additional code
- Risk adjustment
- CMS-HCC V22 category 40 · RxHCC V08 category 84
Billable · FY2027A valid, specific ICD-10-CM code, reportable for dates of service in FY2027.
What you need to know
Source: CMS/NCHS Official ICD-10-CM tabular notes, quoted. From the CMS/NCHS tabular list for the release in force. A note the category or block publishes applies to this code too; the Instructions section marks which is which.
- Use additional codeReport with this code when documented
- code to identify associated manifestations
- Excludes1Never report with this code
- IncludesWhat this code covers
- autoimmune disease NOS
- collagen (vascular) disease NOS
- systemic autoimmune disease
- systemic collagen (vascular) disease
Most relevant related codes MedCoder-derived
Read off the official notes above and this code’s own position in the tabular list. Which to report is a documentation question; Compare shows the two side by side.
Inpatient Payment Groups (MS-DRG)
Potential MS-DRG participation — not a DRG assignment.
MS-DRGs this diagnosis helps define, as principal or secondary, per the CMS ICD-10-CM/PCS MS-DRG Definitions Manual v44, Appendix B.
- MS-DRG 545 — CONNECTIVE TISSUE DISORDERS WITH MCC (MDC 08)
- MS-DRG 546 — CONNECTIVE TISSUE DISORDERS WITH CC (MDC 08)
- MS-DRG 547 — CONNECTIVE TISSUE DISORDERS WITHOUT CC/MCC (MDC 08)
A diagnosis appearing in a group's logic does not by itself determine the DRG assigned to a stay; the grouper uses the full claim.
Coding instructions
Official Tabular Instructional Notes: the inclusion, exclusion and sequencing notes published for M35.03 in the official ICD-10-CM tabular list, quoted as published.
Source: CMS/CDC — ICD-10-CM Tabular ListRelease: FY2027Effective: October 1, 2026
Trace:FY2027 changesChange historyRelease, file and checksum
Notes without a marker are published on M35.03 itself; “inherited from” names the category or block whose note applies here.
Includes
Conditions the official ICD-10-CM tabular list includes under this code.
- autoimmune disease NOS
- collagen (vascular) disease NOS
- systemic autoimmune disease
- systemic collagen (vascular) disease
Source: inherited from M30-M36
Excludes1 — Not Coded Here
Conditions generally not reported together with this code (Excludes1) -- an error unless the two conditions are documented as unrelated to each other (ICD-10-CM Official Guidelines, Section I.A.12.a).
- dry mouth, unspecified (R68.2) inherited from M35.0Compare M35.03 vs R68.2 →
- reactive perforating collagenosis (L87.1) inherited from M35Compare M35.03 vs L87.1 →
- autoimmune disease, single organ or single cell-type -code to relevant condition category inherited from M30-M36
Use Additional Code
Supplementary codes the tabular list directs you to add.
- Use additional code to identify associated manifestations
Source: inherited from M35.0
Coder workflow for M35.03
MedCoder structured workflow — derived from this code’s own official record
Before you code M35.03
- M35.03’s title joins a condition with an associated condition or complication. Confirm each component is documented. Where the classification presumes the link through the “with” convention, only a provider statement that the conditions are unrelated defeats it. A combination code is assigned only when it fully identifies the documented conditions; a required second code for the stage, type or manifestation is still reported when the notes ask for it (Guidelines I.B.9, I.A.15).
- Check the Excludes1 note: if the documentation supports a condition named there, do not simply proceed with M35.03. Excludes1 conditions are not reported together with this code unless the record shows they are unrelated (Guidelines I.A.12.a).
Choose the right path
- Does the documentation support a condition named in M35.03’s Excludes1 note?
Yes → Do not simply proceed: review the excluded code. Both are reported only when the record shows the two conditions are unrelated.
No → Continue.
Consider M35.03. Then work the Use Additional Code note, and confirm the code is valid for the date of service in the Verify section.
Documentation check
- The provider’s diagnostic statement
- Codes are assigned from the provider’s documented diagnosis, not from clinical criteria, test values or a medication list (Guidelines I.A.19).
- The associated condition or complication
- Whether the associated condition the title names is documented; the “with” convention presumes some links, and a provider statement that the conditions are unrelated defeats it (Guidelines I.A.15).
- The conditions the Use Additional Code note names
- Reported with this code when documented; a conditional instruction (“if applicable”, “if known”) applies only when the record supports it.
Official instructions as workflow
Excludes1 — check before selecting M35.03(3 notes)
Coding workflow: If the documentation supports a condition named in this note, do not simply proceed with M35.03: the two are not reported together. The one exception is when the record shows the two conditions are unrelated to each other.
See the official tabular notes · Guidelines I.A.12.a
Use Additional Code — after identifying M35.03(1 note)
Coding workflow: Check whether the documentation supports the additional code(s) the note names, and report them with M35.03 when it does. Where the instruction is conditional (“if applicable”, “if known”), it applies only when the record documents the condition.
See the official tabular notes · Guidelines I.A.13
Coding decision scenarios
Pattern scenarios for this code’s structure — decision rules, not clinical cases
Documentation: Both the condition M35.03 describes and a condition named in its Excludes1 note are documented for the same encounter.
Coding question: Can both codes be reported?
Path: Review the Excludes1 note and the excluded code, and look for a provider statement on whether the two conditions are related.
Reason: Excludes1 means the two are not coded together; the exception is when the record shows the conditions are unrelated to each other (Guidelines I.A.12.a).
Documentation: Only one of the components this code’s title joins is documented.
Coding question: Is M35.03 supported?
Path: Review the code for the documented component on its own.
Reason: A combination code is assigned only when it fully identifies the documented conditions; otherwise the documented component takes its own code (Guidelines I.B.9).
Documentation: A condition the Use Additional Code note names is documented.
Coding question: Is a second code reported with M35.03?
Path: Review the Use Additional Code note and the code it names.
Reason: The additional code is reported when the record documents the condition; a conditional instruction applies only when its condition is met (Guidelines I.A.13).
Every row is derived from this code’s own record — its title, tabular notes, 7th-character family and same-category siblings — with fixed MedCoder wording; nothing is inferred about a patient. The official notes and guideline text are in the sections each row links to, and they control.
Coding context
Guidelines, coding notes, decision aids, relationships (with MS-DRG and CCSR classification), hierarchy, HCC, coverage and the context map: what a coder reaches for after the core. Each section names whether it is official source data, a MedCoder-derived relationship or MedCoder editorial.
Code Overview
MedCoder summary Summary composed by MedCoder from this code's official ICD-10-CM record. The tabular instructional notes themselves appear verbatim below.
Verify Before Coding
- CC as a secondary diagnosis (FY2027). Can raise the stay's MS-DRG severity tier.
From the code registry, the Medicare Code Editor, and the MS-DRG Definitions Manual. Check it against a full claim in Claim Check.
Relationships & Classification
MedCoder-derived relationships — computed from published CMS and AHRQ datasets
Other codes that name M35.03 or its code family, from the CMS ICD-10-CM tabular instructional notes. Tabular-note edges are stored at the code family level that carries each note.
Referenced by 9 Excludes1 notes across 5 chapters: G73.7 — Myopathy in diseases classified elsewhere, K11.8 — Other diseases of salivary glands (via M35.0.-), L94 — Other localized connective tissue disorders (via M35.-), M60 — Myositis, M60-M63 — Disorders of muscles (M60-M63), M61 — Calcification and ossification of muscle, M62 — Other disorders of muscle, M63 — Disorders of muscle in diseases classified elsewhere, R68.2 — Dry mouth, unspecified (via M35.0.-).
These codes’ tabular lists mark this diagnosis as mutually exclusive — not reported together unless the conditions are documented as unrelated.
Referenced by 2 Excludes2 notes across 2 chapters: I77 — Other disorders of arteries and arterioles (via M35.-), J82 — Pulmonary eosinophilia, not elsewhere classified (via M35.-).
These codes’ tabular lists name this diagnosis as distinct — both may be reported when both are documented.
Referenced by 1 Code First instruction: J84.170 — Interstitial lung disease with progressive fibrotic phenotype in diseases classified elsewhere (via M35.-).
Each of these codes carries a Code First note naming this condition — when that code is reported, THIS code is sequenced first, ahead of it.
Referenced by 1 Code Also instruction: I27.21 — Secondary pulmonary arterial hypertension (via M35.0.-).
These codes suggest coding this condition alongside when both are present.
MS-DRG Grouper Relationships (FY2027)
Potential MS-DRG participation — not a DRG assignment.
FY2027 MS-DRG: CC — Complication or Comorbidity. Reported as a secondary diagnosis, this code raises the stay's MS-DRG severity tier — except when the principal diagnosis is one of 113 clinically related codes on its CMS exclusion list.
Named in the grouper logic of 3 MS-DRGs: DRG 545 (MDC 08), DRG 546 (MDC 08), DRG 547 (MDC 08).
From the CMS MS-DRG Definitions Manual (Appendices B and C). Actual DRG assignment depends on the complete claim.
Clinical classification (AHRQ CCSR):MUS024 — Systemic lupus erythematosus and connective tissue disorders (default); NVS018 — Myopathies.
Clinical Classifications Software Refined (CCSR) for ICD-10-CM Diagnoses. Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality.
Related Codes
Same clinical process (MS-DRG)
Acts as CC — raises the severity of other admissions. CMS groups these diagnoses into one MS-DRG principal-diagnosis exclusion process — a CC/MCC on this list never raises severity when the principal diagnosis is also on it.
G71.19 — Other specified myotonic disorders, G71.20 — Congenital myopathy, unspecified, G71.21 — Nemaline myopathy, G71.220 — X-linked myotubular myopathy, G71.228 — Other centronuclear myopathy, G71.29 — Other congenital myopathy, G71.3 — Mitochondrial myopathy, not elsewhere classified, G71.8 — Other primary disorders of muscles, G71.9 — Primary disorder of muscle, unspecified, G72.0 — Drug-induced myopathy, G72.1 — Alcoholic myopathy, G72.2 — Myopathy due to other toxic agents, G72.3 — Periodic paralysis, G72.81 — Critical illness myopathy, G72.89 — Other specified myopathies, G72.9 — Myopathy, unspecified, G96.9 — Disorder of central nervous system, unspecified, G98.0 — Neurogenic arthritis, not elsewhere classified, G98.8 — Other disorders of nervous system, M35.07 — Sjögren syndrome with central nervous system involvement, +92 more
Same clinical category (CCSR)
AHRQ's Clinical Classifications Software groups these diagnoses under the same clinical categories (Systemic lupus erythematosus and connective tissue disorders, Myopathies).
M34.1 — CR(E)ST syndrome, M34.2 — Systemic sclerosis induced by drug and chemical, 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, M35.00 — Sjögren syndrome, unspecified, M35.01 — Sjögren syndrome with keratoconjunctivitis, M35.02 — Sjögren syndrome with lung involvement, M35.04 — Sjögren syndrome with tubulo-interstitial nephropathy, 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, M35.09 — Sjögren syndrome with other organ involvement, M35.0A — Sjögren syndrome with glomerular disease, M35.0B — Sjögren syndrome with vasculitis, M35.0C — Sjögren syndrome with dental involvement, M35.1 — Other overlap syndromes, +145 more
Same Index main term, other category
The ICD-10-CM Index to Diseases and Injuries files this code under the main terms “Myopathy”, “Keratoconjunctivitis”; these codes share that main term but sit in a different category of the Tabular List.
G71.9 — Primary disorder of muscle, unspecified (primary), G72.0 — Drug-induced myopathy (in, drugs), G72.1 — Alcoholic myopathy (alcoholic), G72.2 — Myopathy due to other toxic agents (toxic), G72.49 — Other inflammatory and immune myopathies, not elsewhere classified (immune NEC), G72.81 — Critical illness myopathy (intensive care), G72.89 — Other specified myopathies (specified NEC), G72.9 — Myopathy, unspecified, H05.82 — Myopathy of extraocular muscles (extraocular muscles), H16.20 — Unspecified keratoconjunctivitis, H16.21 — Exposure keratoconjunctivitis (exposure), H16.22 — Keratoconjunctivitis sicca, not specified as Sjögren's (sicca, not Sjögren's), H16.23 — Neurotrophic keratoconjunctivitis (neurotrophic), H16.25 — Phlyctenular keratoconjunctivitis (phlyctenular), H16.26 — Vernal keratoconjunctivitis, with limbar and corneal involvement (vernal), H16.29 — Other keratoconjunctivitis (specified type NEC), M30.0 — Polyarteritis nodosa (in, polyarteritis nodosa), M31.6 — Other giant cell arteritis (in, giant cell arteritis), M32.19 — Other organ or system involvement in systemic lupus erythematosus (in, systemic lupus erythematosus), M34.82 — Systemic sclerosis with myopathy (in, scleroderma), +35 more
Lab tests where this diagnosis supports Medicare coverage (NCD)
Medicare's National Coverage Determination (NCD) program lists this diagnosis as medical justification for these lab tests.
Contextual Map
Every relationship of M35.03 in one view: hierarchy, official tabular instructions in both directions, clinical classification, risk adjustment, MS-DRG participation, index terms and change history — each edge carrying the CMS source it derives from.
Run M35.03 with these 12 related codes in Claim Check
Hierarchy
- M00-M99 — Chapter 13: Diseases of the Musculoskeletal System and Connective Tissue (M00-M99) (M00-M99)[Hierarchy]— CMS ICD-10-CM tabular structure · icd10cm-fy2027
- M30-M36 — Systemic connective tissue disorders[Hierarchy]— CMS ICD-10-CM tabular structure · icd10cm-fy2027
Referenced by Excludes1 notes (9)
- G73.7 — Myopathy in diseases classified elsewhere[Excludes1]: “Sjögren syndrome (M35.03)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2027
- K11.8 — Other diseases of salivary glands[Excludes1](via M35.0.-): “Sjögren syndrome (M35.0-)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2027
- L94 — Other localized connective tissue disorders[Excludes1](via M35.-): “systemic connective tissue disorders (M30-M36)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2027
- M60 — Myositis[Excludes1]: “myopathy in Sjögren's syndrome (M35.03)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2027
- M60-M63 — Disorders of muscles (M60-M63)[Excludes1]: “myopathy in Sjögren's syndrome (M35.03)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2027
- M61 — Calcification and ossification of muscle[Excludes1]: “myopathy in Sjögren's syndrome (M35.03)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2027
- M62 — Other disorders of muscle[Excludes1]: “myopathy in Sjögren's syndrome (M35.03)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2027
- M63 — Disorders of muscle in diseases classified elsewhere[Excludes1]: “myopathy in Sjögren's syndrome (M35.03)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2027
- and 1 more
Referenced by Excludes2 notes
- I77 — Other disorders of arteries and arterioles[Excludes2](via M35.-): “collagen (vascular) diseases (M30-M36)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2027
- J82 — Pulmonary eosinophilia, not elsewhere classified[Excludes2](via M35.-): “pulmonary eosinophilia due to systemic connective tissue disorders (M30-M36)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2027
Referenced by Code First instructions
- J84.170 — Interstitial lung disease with progressive fibrotic phenotype in diseases classified elsewhere[Code First](via M35.-): “systemic connective tissue disorders (M30-M36)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2027
Referenced by Code Also instructions
- I27.21 — Secondary pulmonary arterial hypertension[Code Also](via M35.0.-): “Sjögren syndrome (M35.0-)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2027
Clinical classification (CCSR)
- MUS024 — Systemic lupus erythematosus and connective tissue disorders[CCSR]— AHRQ CCSR for ICD-10-CM Diagnoses (HCUP)
- NVS018 — Myopathies[CCSR]— AHRQ CCSR for ICD-10-CM Diagnoses (HCUP)
MS-DRG Grouper
- CC — Complication or Comorbidity [MS-DRG severity]: “As a secondary diagnosis this code can raise the stay's MS-DRG severity tier (CC).”— CMS MS-DRG Definitions Manual (Appendix C) · FY2027
- DRG 545 — CONNECTIVE TISSUE DISORDERS WITH MCC[MS-DRG]: “CONNECTIVE TISSUE DISORDERS WITH MCC (MDC 08)”— CMS MS-DRG Definitions Manual (Appendix B) · FY2027
- DRG 546 — CONNECTIVE TISSUE DISORDERS WITH CC[MS-DRG]: “CONNECTIVE TISSUE DISORDERS WITH CC (MDC 08)”— CMS MS-DRG Definitions Manual (Appendix B) · FY2027
- DRG 547 — CONNECTIVE TISSUE DISORDERS WITHOUT CC/MCC[MS-DRG]: “CONNECTIVE TISSUE DISORDERS WITHOUT CC/MCC (MDC 08)”— CMS MS-DRG Definitions Manual (Appendix B) · FY2027
MDC crossing
- MDC 08 — Diseases and Disorders of the Musculoskeletal System and Connective Tissue[MDC crossing]: “Diseases and Disorders of the Musculoskeletal System and Connective Tissue — the grouper's crossing between diagnoses and procedures: a principal diagnosis sets the MDC, and same-MDC procedures move the stay to its surgical DRGs. 18,439 same-MDC procedures group here; browse them on the MDC page.”— CMS MS-DRG Definitions Manual · FY2027
Index entries
- Myopathy, in (due to), sicca syndrome[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
- Myopathy, in (due to), Sjögren's syndrome[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
- Syndrome, Sjögren, with, myopathy[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
Nearest codes (25)
- M35 — Other systemic involvement of connective tissue[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2027
- M35.0 — Sjögren syndrome[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2027
- M35.00 — Sjögren syndrome, unspecified[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2027
- M35.01 — Sjögren syndrome with keratoconjunctivitis[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2027
- M35.02 — Sjögren syndrome with lung involvement[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2027
- M35.04 — Sjögren syndrome with tubulo-interstitial nephropathy[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2027
- M35.05 — Sjögren syndrome with inflammatory arthritis[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2027
- M35.06 — Sjögren syndrome with peripheral nervous system involvement[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2027
- and 17 more
Change history (3)
- FY2022 — Description revised[Change history]— CMS release files (code change ledger) · icd10cm-fy2022
- and 2 more
Reference
Index terms and tables, published questions and FAQ, every source behind this page with its release and checksum, the date-of-service check and the complete change history.
Indexed Clinical Terms (3)
Official source data — entries quoted as published, in the Index’s own lookup phrasing
Clinical term phrases from the official ICD-10-CM Index to Diseases and Injuries that map to this code. These are alphabetic-index entries shown as the Index writes them — lookup phrasing, not necessarily the wording of a final diagnosis.
Sources for this page
Codes, titles, notes, index terms and mappings on this page are transcribed from the datasets below. Relationships MedCoder computed and text MedCoder wrote are labelled where they appear.
- Code, title, tabular notes and index terms Official source data
- CMS/CDC ICD-10-CM FY2027 tabular list, index and tables, effective October 1, 2026 Release, file and checksum · Publisher’s page
- Claim edits Official source data
- CMS Definitions of Medicare Code Edits — v44.0 (October 2026) Release, file and checksum · Publisher’s page
- Inpatient payment groups Official source data
- CMS MS-DRG Definitions Manual (incl. Appendix B diagnosis index, Appendix C CC/MCC list, Appendix E procedure index) and IPPS Final Rule tables — v44 Release, file and checksum · Publisher’s page
- Change history and date-of-service validity Official source data
- CMS ICD-10-CM release addenda, ingested release by release into the change ledger Release, file and checksum · Publisher’s page
- Comparisons, relationships and the contextual map MedCoder-derived relationship
- Computed by MedCoder from the tabular notes and tables above; every derived relationship is marked as derived where it appears
- Summary and FAQ answers MedCoder editorial explanation
- Written by MedCoder to explain the sources above: drafted with AI assistance, checked by a person against the release files, and labelled as MedCoder text where it appears. Not official text.
Current data releases:ICD-10-CM FY2027 · ICD-10-PCS FY2027 · HCPCS October 2026 · MS-DRG v44 · Medicare Code Editor v44.0 · NCCI PTP Q4 2026 · MUE Q4 2026 · NCD code lists 2026-01 · LCD export September 28, 2026 · All releases and sources
Labels on this page: Official source data · MedCoder-derived relationship · MedCoder editorial explanation. How to read the labels · All data sources and release dates · CMS coding rules
Cite this page
Reference this page in a research guide, syllabus or article. The release is included because code content changes each year.
MedCoder.ai. "M35.03 — Sjögren syndrome with myopathy." ICD-10-CM FY2027. https://medcoder.ai/icd10/code/m35.03-sjogren-syndrome-with-myopathy
Change history
- FY2022 — October 1, 2021Description revisedSicca syndrome with myopathy → Sjogren syndrome with myopathyFY2022 changes
- FY2022 — October 1, 2021Short description revisedSicca syndrome with myopathy → Sjogren syndrome with myopathyFY2022 changes
- FY2016 — October 1, 2015In the code set at ICD-10-CM adoptionSicca syndrome with myopathy
Nearest Codes in This Family
Official ICD-10-CM classifications closest to M35.03 in its code family, with their registry titles.
- M35 — Other systemic involvement of connective tissue
- M35.0 — Sjögren syndrome
- M35.00 — Sjögren syndrome, unspecified
- M35.01 — Sjögren syndrome with keratoconjunctivitis
- M35.02 — Sjögren syndrome with lung involvement
- M35.04 — Sjögren syndrome with tubulo-interstitial nephropathy
- 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