Modified classification of autoimmune adrenal insufficiency

Objectives: to modify the classification of autoimmune adrenal insufficiency (AAI).

Methods: n=8 patients with early stages (potential and latent) of AAI were included (table 1).

Results: Based on the analysis of clinical and laboratory data of patients, a modified classification of AAI has been developed (table 2).

Table 1. Characteristics of patients
Characteristics	Patients
	№1	№2	№3	№4	№5	№6	№7	№8
Sex (F/M)	F	M	F	M	M	F	F	F
Age, years	25	26	19	50	18	40	38	39
Antibodies to P450c21, U/ml (RI < 0,4)	2,940	92,670	0,604	71,976	12,858	55,623	23,604	35,508
ACTH, pg/ml (RI 7,2-63,3)	84	79	51	501	81	1077	101	21
Basal cortisol, nmol/l (RI 171-536)	402,8	477,7	576,4	157,7	482,3	342,7	420,4	560,7
Maximum cortisol during insulin tolerance test, nmol/l (RI ≥ 500)	481,6	509,4	-	141,4	497,2	350,3	690,4	-
Aldosterone, pmol/l (RI 69,8-1085,8)	121	74	207	73,4	237	350	112	732
Renin, IU/l (RI 2,8-39,9)	> 500	125	26	331	164	379	> 500	18
DHEA-S, mcmol/l	0,1*	1,6**	3,1*	3,0**	2,1**	0,7***	0,5*	3,2*
Stage of AAI	lAAI	lAAI	pAAI	lAAI	lAAI	lAAI	lAAI	pAAI
* RI 1,65-11 RI 1,2-13,4* RI 0,26-6,68 Notes: F – female; M – male; P450c21 – 21-hydroxylase; ACTH – adrenocorticotropic hormone; DHEA-S – dehydroepiandrosterone sulfate; RI – reference interval; AAI – autoimmune adrenal insufficiency; l – latent; P – potential.

Table 2. Modified classification of AAI
Stages	AB to P450c21	ACTH	Cortisol basal	Cortisol during insulin tolerance test, nmol/l	R	A	D-S¹	Clinics of AAI
pAAI	↑	N	N	≥ 500	N	N	N	-
lAAI
-substage 1	↑	N/↑	N/↓ (but ≥ 140 nmol/l)	≥ 500	↑	N/↓	↓	±
- substage 2	↑	N/↑	N/↓ (but ≥ 140 nmol/l)	< 500	↑	N/↓	↓	±
mAAI	↑²/N³	↑↑	↓↓ (< 140 nmol/l)	< 500	↑↑²	N/↓	↓	+²
Notes: AAI – autoimmune adrenal insufficiency; AB – antibodies; P450c21 – 21-hydroxylase; ACTH – adrenocorticotropic hormone; R – renin; A – aldosterone; D-S – dehydroepiandrosterone sulfate; N – norm (within the laboratory reference interval); ↑ – increased; ↑↑ – significantly increased; ↓ – decreased; ↓↓ – significantly reduced; l – latent; P – potential; m – manifest.¹ An additional diagnostic criterion. It is advisable to conduct the study only in women aged 18-40 years.² At the onset of the disease.³ With a long course of the disease.

Conclusion: It is recommended to introduce a modified classification of AAI in clinical practice.

Endocrine Abstracts