ICD-10-CM · Other

M35.01

Sjögren syndrome with keratoconjunctivitis sicca — the specific subcode for Sjögren-related dry eye and corneal inflammation confirmed as autoimmune in etiology.

Verified May 8, 2026 · 6 sources ↓

Status
Billable
Chapter
13
Related CPT
5
Region
Other
Drawn from CDCICD10DataAAPCAaoThe-rheumatologist

Documentation tips

What should appear in the chart to support M35.01.

Source · Editorial brief grounded in 6 cited references ↓

  • Explicitly document 'Sjögren syndrome' as the etiology of the keratoconjunctivitis — 'KCS due to Sjögren's' or equivalent phrasing is required; generic 'dry eye' does not support M35.01.
  • Record objective ocular findings: Schirmer test results, ocular surface staining score (van Bijsterveld or Oxford scale), tear break-up time, or slit-lamp findings of corneal epitheliopathy.
  • Document serologic findings that support the Sjögren diagnosis — anti-SSA/Ro, anti-SSB/La, ANA titer, or salivary gland biopsy result — to anchor the systemic autoimmune context.
  • If lip/salivary gland involvement, inflammatory arthritis, or other system manifestations are also present, document each separately so additional M35.0x subcodes can be stacked per tabular instructions.
  • Note prior treatments (cyclosporine drops, lifitegrast, punctal plugs, artificial tears) to support medical necessity and chronicity when submitting to payers.

Related CPT procedures

Procedure codes commonly billed with M35.01. Linking the right diagnosis to the right procedure is what establishes medical necessity.

Source · CMS LCDs · AAOS specialty guidance · claims-pattern analysis

Common coding pitfalls

The recurring mistakes coders make with M35.01 and adjacent codes.

Source · Editorial brief grounded in CDC ICD-10-CM tabular guidance, AAOS coding references, and cited references ↓

  • Coding both M35.01 and H16.22 (Keratoconjunctivitis sicca, not specified as Sjögren's) on the same claim — the Excludes1 relationship prohibits this; payers will deny the combination.
  • Using M35.0 (parent, non-billable) instead of the specific subcode M35.01 — always code to the highest level of specificity for a billable encounter.
  • Appending R68.2 (dry mouth, unspecified) alongside M35.01 — the Excludes1 note at M35.0 blocks this pairing.
  • Failing to add manifestation codes when other Sjögren-related organ involvement is documented — the tabular 'Use additional code' instruction is mandatory, not optional.
  • Confusing the old description 'Sicca syndrome with keratoconjunctivitis' (pre-FY2022) with a separate code — it is the same M35.01 code, just renamed.

Clinical context

Source · Editorial summary grounded in 6 cited references ↓

M35.01 captures Sjögren syndrome when keratoconjunctivitis sicca (KCS) is the documented manifestation — meaning the patient has both a confirmed or working diagnosis of Sjögren syndrome AND ocular surface disease (dry eye with corneal/conjunctival involvement) attributable to it. This code sits under parent M35.0 (Sicca syndrome [Sjögren]) and is the most granular option when ocular involvement is the presenting or concurrent feature.

The ICD-10 tabular instruction at M35.0 includes a 'Use additional code to identify associated manifestations,' so you must code beyond M35.01 when other organ systems are involved — for example, adding M35.02 for lip/salivary gland involvement or M35.03 for myopathy if those are separately documented. There is also a hard Excludes1 note between M35.01 and H16.22 (Keratoconjunctivitis sicca, not specified as Sjögren's): submit one or the other, never both. R68.2 (dry mouth, unspecified) is similarly excluded at the parent level — don't bolt it on as an addendum code.

M35.01 replaced the legacy description 'Sicca syndrome with keratoconjunctivitis' effective FY2022. Encounters coded prior to October 1, 2021 may appear in legacy records under that descriptor. For inpatient stays, M35.01 maps to MS-DRG 545/546/547 (Connective tissue disorders with MCC/CC/without CC-MCC), so accurate CC/MCC assignment affects DRG weight — document comorbidities thoroughly.

Sibling codes

Other billable codes under M35.0 (laterality / anatomic variants).

Frequently asked questions

Source · Generated from the editorial pipeline, verified against 6 cited references ↓

01Can I bill M35.01 and H16.22 together on the same claim?
No. H16.22 carries a Type 1 Excludes note back-referencing M35.01, meaning they cannot be reported together. Use M35.01 when Sjögren syndrome is the documented etiology; use H16.22 only when KCS is NOT attributed to Sjögren's.
02What is the difference between M35.0 and M35.01?
M35.0 is the non-billable parent code for Sicca syndrome (Sjögren). M35.01 is the billable subcode specifying keratoconjunctivitis as the manifestation. Always use the subcode — submitting M35.0 alone will trigger specificity edits.
03Do I need to code the keratoconjunctivitis separately in addition to M35.01?
No. M35.01 is a combination code that captures both the Sjögren syndrome and the keratoconjunctivitis in a single code. Additional ophthalmic codes for the same KCS are not required and the Excludes1 on H16.22 actively prohibits double-coding it.
04What if the patient has both ocular and oral manifestations of Sjögren syndrome?
Stack codes per the tabular 'Use additional code' instruction. Report M35.01 for the keratoconjunctivitis and M35.02 for salivary gland involvement if both are documented. Each subcode is independently billable.
05Is M35.01 appropriate for a rheumatology encounter or only ophthalmology?
It is appropriate in any setting where Sjögren syndrome with keratoconjunctivitis is the documented diagnosis — rheumatology, ophthalmology, primary care, or hospital inpatient. The diagnosing provider's specialty does not restrict its use.
06How did the code description change in FY2022?
Effective October 1, 2021, the description changed from 'Sicca syndrome with keratoconjunctivitis' to 'Sjogren syndrome with keratoconjunctivitis.' The code number M35.01 did not change — only the label was updated to eliminate the ambiguous 'sicca syndrome' terminology.
07What MS-DRGs does M35.01 map to for inpatient encounters?
M35.01 maps to MS-DRG 545 (Connective tissue disorders with MCC), 546 (with CC), and 547 (without CC/MCC) under MS-DRG v43.0. Thorough comorbidity documentation determines which tier applies and directly affects reimbursement weight.

Mira AI Scribe

Mira's AI scribe captures the clinician's documented Sjögren diagnosis, objective ocular findings (Schirmer score, staining grade, TBUT), serologic markers (anti-SSA/Ro, anti-SSB/La), and any concurrent Sjögren manifestations — preventing unspecified M35.0 usage, the M35.01/H16.22 Excludes1 denial, and missing stacked manifestation codes that affect DRG weight.

See how Mira captures M35.01 documentation

Related ICD-10 codes

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