N04 ICD-10-CM Code: Nephrotic syndrome

Coding instructions

Official Tabular Instructional Notes: the inclusion, exclusion and sequencing notes published for N04 in the official ICD-10-CM tabular list, quoted as published.

Source: CMS/CDC — ICD-10-CM Tabular ListRelease: FY2026Effective: October 1, 2025

Notes without a marker are published on N04 itself; “inherited from” names the category or block whose note applies here.

Coder workflow for N04

MedCoder structured workflow — derived from this code’s own official record

Before you code N04

1. N04 is not reportable as written. Select the more specific code beneath it that the documentation supports. Codes are reported to the highest level of specificity the classification provides (Guidelines I.B.2).

   ReviewN04.0, N04.1, N04.2, N04.3, N04.4, N04.5, N04.6, N04.7, N04.8, N04.9, N04.A, N04.B

   See the relationships section · Guide: How to choose an ICD-10-CM code →

2. Check the Excludes1 note: if the documentation supports a condition named there, do not simply proceed with N04. Excludes1 conditions are not reported together with this code unless the record shows they are unrelated (Guidelines I.A.12.a).

   See the official tabular notes

Choose the right path

1. Does the documentation support one of the more specific codes beneath N04?
   Yes → Select that code and continue the checks below on its own page.
   No → N04 cannot be reported as written; query for the specificity its subcategory needs.

   ReviewN04.0, N04.1, N04.2, N04.3, N04.4, N04.5, N04.6, N04.7, N04.8, N04.9, N04.A, N04.B

2. Does the documentation support a condition named in N04’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.

   ReviewI12

Consider N04. Then review the Code Also 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).

Official instructions as workflow

• Excludes1 — check before selecting N04(1 note)

  Coding workflow: If the documentation supports a condition named in this note, do not simply proceed with N04: the two are not reported together. The one exception is when the record shows the two conditions are unrelated to each other.

  CompareI12

  See the official tabular notes · Guidelines I.A.12.a

• Code Also — related condition(1 note)

  Coding workflow: Review the related condition when both are documented and the instruction applies. A Code Also note does not fix sequencing; the order follows the circumstances of the encounter.

  See the official tabular notes · Guidelines I.A.17

Coding decision scenarios

Pattern scenarios for this code’s structure — decision rules, not clinical cases

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.

Don’t confuse N04 with…

MedCoder analysis — pairs from this code’s official notes, distinctions written by MedCoder

The codes most likely to be picked instead of N04 — named in its own official notes, or sharing its laterality or encounter family — with what separates them and which documentation moves the case. Each links to the full comparison.

• I12— Excludes1 conflict (Excludes1 — generally not reported together)

  Not coded here — the tabular list excludes this pair for the same encounter.

  Not reported together with this code, except where the two conditions are documented as unrelated.

  What moves you there: Documentation of hypertensive chronic kidney disease moves the case to I12; the two are not reported together unless documented as unrelated.

  Full comparison: N04 vs I12 →

Relationships are read from this code's own official notes, its laterality siblings and its encounter family; the wording of each distinction is MedCoder editorial, and the documentation named in each “what moves you there” line is the official note phrase or sibling title itself. Source: CMS ICD-10-CM tabular instructional notes; ICD-10-CM Official Guidelines, Section I.A.12.a.

Verify Before Coding

• Not billable as written — a more specific code is required: N04.0, N04.1, N04.20, N04.21, N04.22.

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 structured relationships — computed from published CMS and AHRQ datasets

Other codes that name N04 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 1 Excludes1 note: Q63 — Other congenital malformations of kidney.

These codes’ tabular lists mark this diagnosis as mutually exclusive — not reported together unless the conditions are documented as unrelated.

Contextual Map

Every relationship of N04 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 N04 with these 5 related codes in Claim Check

Hierarchy

• N00-N99 — Chapter 14: Diseases of the Genitourinary System (N00-N99) (N00-N99)[Hierarchy]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• N00-N08 — Glomerular diseases[Hierarchy]— CMS ICD-10-CM tabular structure · icd10cm-fy2026

Excludes1

• I12 — Hypertensive chronic kidney disease[Excludes1]: “hypertensive chronic kidney disease (I12.-)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026

Code Also

• N17 — Acute kidney failure[Code Also]: “any associated kidney failure (N17-N19).”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026
• N18 — Chronic kidney disease (CKD)[Code Also]: “any associated kidney failure (N17-N19).”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026
• N19 — Unspecified kidney failure[Code Also]: “any associated kidney failure (N17-N19).”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026

Referenced by Excludes1 notes

• Q63 — Other congenital malformations of kidney[Excludes1]: “congenital nephrotic syndrome (N04.-)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026

Nearest codes (18)

• N04.0 — Nephrotic syndrome with minor glomerular abnormality[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• N04.1 — Nephrotic syndrome with focal and segmental glomerular lesions[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• N04.2 — Nephrotic syndrome with diffuse membranous glomerulonephritis[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• N04.20 — Nephrotic syndrome with diffuse membranous glomerulonephritis, unspecified[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• N04.21 — Primary membranous nephropathy with nephrotic syndrome[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• N04.22 — Secondary membranous nephropathy with nephrotic syndrome[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• N04.29 — Other nephrotic syndrome with diffuse membranous glomerulonephritis[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• N04.3 — Nephrotic syndrome with diffuse mesangial proliferative glomerulonephritis[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• and 10 more

Change history

• FY2016 — In the code set at ICD-10-CM adoption [Change history]— CMS release files (code change ledger) · icd10cm-fy2016

Common coding questions

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 FY2026 tabular list, index and tables, effective October 1, 2025 Release, file and checksum · Publisher’s page

Claim edits Official source data

CMS Definitions of Medicare Code Edits — v43.1 (April 2026) 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 structured 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 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.

Labels on this page: Official source data · MedCoder structured relationship · MedCoder explanation. How to read the labels · All data sources and release dates

Nearest Codes in This Family

Official ICD-10-CM classifications closest to N04 in its code family, with their registry titles.

• N04.0 — Nephrotic syndrome with minor glomerular abnormality
• N04.1 — Nephrotic syndrome with focal and segmental glomerular lesions
• N04.2 — Nephrotic syndrome with diffuse membranous glomerulonephritis
• N04.20 — Nephrotic syndrome with diffuse membranous glomerulonephritis, unspecified
• N04.21 — Primary membranous nephropathy with nephrotic syndrome
• N04.22 — Secondary membranous nephropathy with nephrotic syndrome
• N04.29 — Other nephrotic syndrome with diffuse membranous glomerulonephritis
• N04.3 — Nephrotic syndrome with diffuse mesangial proliferative glomerulonephritis
• N04.4 — Nephrotic syndrome with diffuse endocapillary proliferative glomerulonephritis
• N04.5 — Nephrotic syndrome with diffuse mesangiocapillary glomerulonephritis
• N04.6 — Nephrotic syndrome with dense deposit disease
• N04.7 — Nephrotic syndrome with diffuse crescentic glomerulonephritis
• N04.8 — Nephrotic syndrome with other morphologic changes
• N04.9 — Nephrotic syndrome with unspecified morphologic changes
• N04.A — Nephrotic syndrome with C3 glomerulonephritis
• N04.B — Nephrotic syndrome with immune complex membranoproliferative glomerulonephritis (IC-MPGN)
• N04.B1 — Nephrotic syndrome with idiopathic immune complex membranoproliferative glomerulonephritis (IC-MPGN)
• N04.B2 — Nephrotic syndrome with secondary immune complex membranoproliferative glomerulonephritis (IC-MPGN)