N01.6 — Rapidly progressive nephritic syndrome with dense deposit disease
ICD-10-CM
Prior Authorization
No Prior Auth Required
Code is covered without prior authorization (high confidence)
Documentation Required
- Document meningococcal immunization at least 2 weeks prior to the first eculizumab dose or a clinical justification that delaying eculizumab posed greater risk than meningococcal infection.
- For PNH initiation, document flow cytometry confirming PNH and either evidence of clinically significant hemolysis or a documented history of a major adverse vascular event (MAVE) from thromboembolism.
- For aHUS initial trial, document absence of signs of STEC-HUS and that TTP was ruled out (e.g., normal ADAMTS13 activity and no inhibitor), or document a plasma exchange trial that failed to produce clinical improvement.
- If continuing eculizumab after the aHUS initial trial, document clinical improvement such as increased platelet count or laboratory evidence of reduced hemolysis.
Key Coverage Criteria
- Eculizumab is covered for treatment of paroxysmal nocturnal hemoglobinuria (PNH) when flow cytometry confirms PNH and there is either evidence of clinically significant hemolysis or a documented history of a major adverse vascular event (MAVE) from thromboembolism.
- Eculizumab is covered for treatment of atypical hemolytic uremic syndrome (aHUS) as an initial trial (generally 6–12 weeks) when the individual has no signs of STEC-HUS and thrombotic thrombocytopenic purpura (TTP) has been ruled out or a trial of plasma exchange did not produce clinical improvement.
- Eculizumab is covered for biopsy-proven dense deposit disease when elevated serum sC5b-9 (sMAC) levels are documented.
- Eculizumab may be covered for other label and off-label indications supported by recognized compendia or the FDA label when dose and frequency conform to FDA labeling or recognized compendia guidance.
- Retroperitoneal ultrasound (CPT 76770, 76775, 76776) is covered when the exam is limited solely to retroperitoneal structures.
- A full (complete) or limited abdominal ultrasound (CPT 76700, 76705, 76706) is covered when the exam includes organs or structures outside the retroperitoneum or when primary findings involve non-retroperitoneal structures (e.g., gallbladder, liver, spleen, common bile duct).
10 Active Policies
A54548 — Billing and Coding: Eculizumab, and Biosimilars: EPYSQLI-eculizumab-aagh, and BKEMV-eculizumab-aeeb
J06
L33394 — Drugs and Biologicals, Coverage of, for Label and Off-Label Uses
J06
A56380 — Billing and Coding: Rituximab
A57189 — Billing and Coding: Serum Magnesium
L34577 — Retroperitoneal Ultrasound
Related Codes
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