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E27.1

Billable

Primary adrenocortical insufficiency

HCC Category Mapping

V28HCC 51Other Significant Endocrine and Metabolic Disorders
0.233
V24HCC 23Other Significant Endocrine and Metabolic Disorders
0.230
ESRDHCC 23Other Significant Endocrine and Metabolic Disorders
0.000
RxHCCHCC 43Other Significant Endocrine and Metabolic Disorders
0.000

What This Code Means

A condition where the adrenal glands do not produce enough hormones, particularly cortisol and aldosterone, due to primary gland failure.

Coding Tips

  • This is primary insufficiency (adrenal gland failure), not secondary to pituitary disease
  • Often associated with autoimmune destruction; document any known etiology for more specific coding if available

Clinical Significance

Primary adrenocortical insufficiency (Addison disease) results from destruction of the adrenal cortex, most commonly by autoimmune adrenalitis in developed countries, causing deficiency of cortisol, aldosterone, and adrenal androgens. Without treatment, this condition is fatal, and even with treatment, patients face lifelong risk of adrenal crisis during physiologic stress.

Documentation Requirements

  • Document the underlying etiology (autoimmune, infectious, hemorrhagic, infiltrative, genetic), cortisol and adrenocorticotropic hormone levels (low cortisol with elevated adrenocorticotropic hormone), adrenocorticotropic hormone stimulation test results, adrenal antibody status, mineralocorticoid and glucocorticoid replacement regimen, and sick-day management plan.

Excludes 1 — Do NOT code together

  • Addison only phenotype adrenoleukodystrophy (E71.528)
  • amyloidosis (E85.-)
  • tuberculous Addison's disease (A18.7)
  • Waterhouse-Friderichsen syndrome (A39.1)

Commonly Confused Codes

Code Hierarchy

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