2027 ICD-10-CM Diagnosis Code E83.822ENPP1 deficiency causing autosomal recessive hypophosphatemic rickets type 2
ICD-10-CM Codes›E00–E89›E70-E88›E83
- Billable — Valid for Submission
- Chronic Condition
E83.822 is a billable ICD-10-CM diagnosis code for ENPP1 deficiency causing autosomal recessive hypophosphatemic rickets type 2. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 642. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Osteomalacia and Other specified and unspecified nutritional and metabolic disorders.
E83.822 does not risk-adjust for Medicare Advantage under the CMS-HCC V28 model. It still risk-adjusts in the PACE (CMS-HCC V22) category 23, ESRD (V21) category 23, ESRD (V24) category 23, and RxHCC Part D (V08) category 43 for payment year 2027.
Code Identity
Code Classification
Medicare Risk Adjustment (HCC)Billing
E83.822 does not risk-adjust for Medicare Advantage under the CMS-HCC V28 model, but it maps to payment categories in the other CMS models shown below.
Source: CMS Payment Year 2027 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to E83.822: its own notes plus those printed at E83, block E70-E88, and Chapter 4. A note printed at a category, block or chapter applies to every code under it.
Excludes1
Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.
Excludes2
Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.
- Ehlers-Danlos syndromes (Q79.6-)
Notes
General instructions on how these codes are used.
- All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Referenced in Other NotesGuidance
Instructional notes printed at other codes that name E83.822, its category, or a range that includes it.
Excludes1 2
These codes carry an Excludes1 note naming E83.822: they are not reported together with it, unless the two conditions are unrelated.
Excludes2 4
These codes carry an Excludes2 note naming E83.822: its condition is not part of those codes, and both may be reported when the patient has both.
- E83.3 Disorders of phosphorus metabolism and phosphatasesdisorders of pyrophosphate metabolism (E83.82-) names E83.82-, which includes this code
- I70.9 Other and unspecified atherosclerosisdisorders of pyrophosphate metabolism (E83.82-) names E83.82-, which includes this code
- Q28.8 Other specified congenital malformations of circulatory systemdisorders of pyrophosphate metabolism (E83.82-) names E83.82-, which includes this code
- Q82.8 Other specified congenital malformations of skindisorders of pyrophosphate metabolism (E83.82-) names E83.82-, which includes this code
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Patient EducationClinical
Rickets
Rickets causes soft, weak bones in children. It usually occurs when they do not get enough vitamin D, which helps growing bones absorb the minerals calcium and phosphorous. It can also happen when calcium or phosphorus levels are too low.
Read the full article at MedlinePlus
Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.
Code History & ChangesHistory
Replacement E83.822 replaces the following previously assigned code(s):
- E83.89 - Other disorders of mineral metabolism
Questions About E83.822Overview
Is E83.822 (Disorders of pyrophosphate metabolism) a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report ENPP1 deficiency causing autosomal recessive hypophosphatemic rickets type 2 on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
What MS-DRG does E83.822 group to?
When ENPP1 deficiency causing autosomal recessive hypophosphatemic rickets type 2 is the principal diagnosis on an inpatient stay, it groups to MS-DRG 642 (inborn and Other Disorders of Metabolism), which carries a relative weight of 1.3226. Higher weights mean higher Medicare reimbursement.
Can E83.822 be reported with a code from E55?
Not as a rule. The Excludes1 note at E83, which applies to E83.822, lists "vitamin D deficiency (E55.-)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).
Does E83.822 risk-adjust for Medicare Advantage payment?
Not for Medicare Advantage: E83.822 maps to no category in the CMS-HCC V28 model. It still risk-adjusts in the PACE (CMS-HCC V22) category 23 (Other Significant Endocrine and Metabolic Disorders), ESRD (V21) category 23 (Other Significant Endocrine and Metabolic Disorders), ESRD (V24) category 23 (Other Significant Endocrine and Metabolic Disorders), and RxHCC Part D (V08) category 43 (Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders).
