ICD-10-CM 2027
ICD-10 code for nephrotic congenital syndrome
Nephrotic syndrome with unspecified morphologic changes
From the official ICD-10-CM alphabetic index entry “Syndrome › nephrotic (congenital)”. Page updated September 29, 2026.
About coding nephrotic congenital syndrome
The ICD-10-CM code for nephrotic congenital syndrome is N04.9 (Nephrotic syndrome with unspecified morphologic changes). The official index lists 13 more specific codes, so check the documentation for details such as type, cause, site or severity before settling on N04.9.
Within N04, choose N04.9 (with unspecified morphologic changes) only when documentation doesn't support a more specific option: with minor glomerular abnormality (N04.0), with focal and segmental glomerular lesions (N04.1), with diffuse membranous glomerulonephritis (N04.2), with diffuse mesangial proliferative glomerulonephritis (N04.3), with diffuse endocapillary proliferative glomerulonephritis (N04.4), with diffuse mesangiocapillary glomerulonephritis (N04.5), with dense deposit disease (N04.6), with diffuse crescentic glomerulonephritis (N04.7) and 3 more.
As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.
In MS-DRG v44.0, it is part of the grouping logic for DRG 698 (Other Kidney and Urinary Tract Diagnoses with MCC, relative weight 1.6398), DRG 699 (Other Kidney and Urinary Tract Diagnoses with CC, relative weight 1.0003) and DRG 700 (Other Kidney and Urinary Tract Diagnoses without CC/MCC, relative weight 0.6741), in MDC 11 (Diseases and Disorders of the Kidney and Urinary Tract), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
More specific nephrotic congenital syndrome codes (official index)
- with
- C3
- glomerulonephritis N04.A — Nephrotic syndrome with C3 glomerulonephritis
- glomerulopathy N04.A — Nephrotic syndrome with C3 glomerulonephritis
- with dense deposit disease N04.6 — Nephrotic syndrome with dense deposit disease
- dense deposit disease N04.6 — Nephrotic syndrome with dense deposit disease
- diffuse
- crescentic glomerulonephritis N04.7 — Nephrotic syndrome with diffuse crescentic glomerulonephritis
- endocapillary proliferative glomerulonephritis N04.4 — Nephrotic syndrome with diffuse endocapillary proliferative glomerulonephritis
- membranous glomerulonephritis N04.20 — Nephrotic syndrome with diffuse membranous glomerulonephritis, unspecified
- mesangial proliferative glomerulonephritis N04.3 — Nephrotic syndrome with diffuse mesangial proliferative glomerulonephritis
- mesangiocapillary glomerulonephritis N04.5 — Nephrotic syndrome with diffuse mesangiocapillary glomerulonephritis
- focal and segmental glomerular lesions N04.1 — Nephrotic syndrome with focal and segmental glomerular lesions
- minor glomerular abnormality N04.0 — Nephrotic syndrome with minor glomerular abnormality
- specified morphological changes NEC N04.8 — Nephrotic syndrome with other morphologic changes
- diabetic
- specified type NEC with diffuse membranous glomerulonephritis N04.29 — Other nephrotic syndrome with diffuse membranous glomerulonephritis
Common questions about nephrotic congenital syndrome ICD-10 codes
What is the ICD-10 code for nephrotic congenital syndrome?
- N04.9 — Nephrotic syndrome with unspecified morphologic changes. More specific codes apply when the documentation supports them: N04.A, N04.A, N04.6, N04.6 and others below.
Is N04.9 billable?
- Yes. N04.9 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can N04.9 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict N04.9 as a principal diagnosis.
Is N04.9 a CC or MCC?
- N04.9 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.