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    OpenPayerBilling CodesGlomerular diseasesICD-10-CM n01-rapidly-progressive-nephritic-syndrome
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    ICD-10-CM Code For Rapidly Progressive Nephritic Syndrome: Group Overview

    The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group N01 includes rapidly progressive nephritic syndromes characterized by acute, severe glomerular inflammation often with crescent formation and rapidly declining renal function. These ICD-10-CM diagnosis codes are used to document acute glomerular injury scenarios, capturing disease severity, organ involvement, and clinical complexity relevant to this group. Accurate coding within the ICD-10-CM N01 group supports appropriate claim adjudication, complete clinical documentation, and appropriate reimbursement.

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    ICD-10-CM N01: Rapidly Progressive Nephritic Syndrome Overview

    Rapidly progressive nephritic syndrome represents a group of glomerular diseases characterized by rapid loss of renal function with nephritic features such as hematuria, proteinuria, and hypertension, often associated with crescent formation on biopsy. These diagnoses target the renal glomeruli and related immune-mediated or systemic processes affecting kidney function. Accurate coding matters for clinical documentation and proper reimbursement because it captures disease severity, acuity, and the need for intensive management.

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    When to Use

    Use a code from this group when a clinician documents a rapidly progressive nephritic syndrome with acute decline in renal function and nephritic features such as hematuria and proteinuria. Codes should be selected when clinical, laboratory, and pathological findings support an acute glomerular inflammatory process requiring urgent management.

    ICD-10-CM Codes

    11 rows

    ICD-10-CM CodeDescription
    N01.0Rapidly progressive nephritic syndrome with minor glomerular abnormality
    N01.1Rapidly progressive nephritic syndrome with focal and segmental glomerular lesions
    N01.2Rapidly progressive nephritic syndrome with diffuse membranous glomerulonephritis
    N01.3Rapidly progressive nephritic syndrome with diffuse mesangial proliferative glomerulonephritis
    N01.4Rapidly progressive nephritic syndrome with diffuse endocapillary proliferative glomerulonephritis
    N01.5Rapidly progressive nephritic syndrome with diffuse mesangiocapillary glomerulonephritis
    N01.6Rapidly progressive nephritic syndrome with dense deposit disease
    N01.7Rapidly progressive nephritic syndrome with diffuse crescentic glomerulonephritis
    N01.8Rapidly progressive nephritic syndrome with other morphologic changes
    N01.9Rapidly progressive nephritic syndrome with unspecified morphologic changes
    N01.ARapidly progressive nephritic syndrome with C3 glomerulonephritis

    DRG Codes

    5 rows

    DRG CodeDescription
    698Other Kidney and Urinary Tract Diagnoses with MCC
    699Other Kidney and Urinary Tract Diagnoses with CC
    700Other Kidney and Urinary Tract Diagnoses without CC/MCC
    791Prematurity with Major Problems
    793Full Term Neonate with Major Problems

    Typical Clinical Scenarios

    • An adult presents emergently with sudden onset severe hematuria, oliguria, facial and peripheral edema, hypertension, and rapidly worsening serum creatinine over days. Urinalysis shows dysmorphic red blood cells and RBC casts, and serologic testing for anti-GBM and ANCA is pending; a renal biopsy demonstrates crescentic glomerulonephritis involving >50% of glomeruli, consistent with a rapidly progressive nephritic process requiring urgent immunosuppressive therapy. A diagnosis from this group is assigned to capture the acute, crescentic glomerular injury causing rapidly progressive renal failure. Typical codes: N01.0, N01.1.
    • A middle-aged patient with known immune complex–mediated glomerulonephritis (eg, postinfectious or lupus-associated) has a subacute worsening of renal function over 2–6 weeks with new gross hematuria, proteinuria in the nephritic range, and rising creatinine despite outpatient management. Nephrology documents progression to crescentic changes on biopsy and escalates therapy; a code from this group is used to indicate the rapidly progressive nephritic transformation of an underlying glomerular disease. Typical codes: N01.2, N01.8.
    • An older patient hospitalized for multisystem vasculitis presents with rapidly declining urine output and azotemia while receiving treatment for systemic disease; urinalysis shows RBC casts and biopsy reveals focal necrotizing crescentic glomerulonephritis as a complication of the systemic vasculitis. A code in this group is assigned to reflect the nephritic rapid progression as a comorbid glomerular complication influencing inpatient management and prognosis. Typical codes: N01.3, N01.7.

    Related Codes

    N00-ACUTE-NEPHRITIC-SYNDROME

    ICD-10-CM Code For Acute Nephritic Syndrome: Group Overview

    N02-RECURRENT-AND-PERSISTENT-HEMATURIA

    ICD-10-CM Code For Recurrent And Persistent Hematuria: Group Overview

    N03-CHRONIC-NEPHRITIC-SYNDROME

    ICD-10-CM Code For Chronic Nephritic Syndrome: Group Overview

    N04-NEPHROTIC-SYNDROME

    ICD-10-CM Code For Nephrotic Syndrome: Group Overview

    N05-UNSPECIFIED-NEPHRITIC-SYNDROME

    ICD-10-CM Code For Unspecified Nephritic Syndrome: Group Overview

    N06-ISOLATED-PROTEINURIA-WITH-LESION

    ICD-10-CM Code For Isolated Proteinuria With Specified Morphological Lesion: Group Overview

    N07-HEREDITARY-NEPHROPATHY-NOT-ELSEWHERE-CLASSIFIED

    ICD-10-CM Code For Hereditary Nephropathy Not Elsewhere Classified: Group Overview

    N08-GLOMERULAR-DISORDERS-IN-DISEASES-CLASSIFIED-ELSEWHERE

    ICD-10-CM Code For Glomerular Disorders In Diseases Classified Elsewhere: Group Overview

    Frequently Asked Questions

    References

    1. 1.CPT® Codes and Descriptions. AAPC. Retrieved Aug 11, 2026.Maintains the CPT code set's official descriptions and guidelines, licensed from the American Medical Association, used as the definitional source for CPT codes on this page.
    2. 2.HCPCS Quarterly Update. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's quarterly release of HCPCS Level II codes and short/long descriptors, the source for HCPCS code definitions and update history.
    3. 3.ICD-10-CM Full Code List. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's complete ICD-10-CM diagnosis code list, used to cross-reference diagnosis codes associated with this procedure or service.
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