Table 1.
Characteristics of 4 patients diagnosed with previously unknown AI among women with adrenal hypoandrogenemia*
| Patient | Age (years) | Diagnoses | Laboratory | Final diagnosis | ||||||
|---|---|---|---|---|---|---|---|---|---|---|
| Primary | Other | Immune | FSH (mIU/ML) | AMH (ng/mL) | Androgens | ACTH (pg/mL) | Cortisol (ug/dL) | |||
| 1 | 28 | POI1 | Hypothyroid | +TPO2 | 30.0 | 3.04 | TT 7.0 ng/dL | 464.7 | 8.8 | Primary AI |
| + TG3 | 14.4 | 2.12 | TT ud6 | |||||||
| Celiac | + DG4 | DHEA ud | ||||||||
| + TG5 | DHEAS ud | |||||||||
| 2 | 30 | PCOS | SLE7 | 8.0 | 2.68 | FT 0.4 pg/mL | ** | Likely iatrogenic AI | ||
| HNA8 | TT 17.0 ng/dL | |||||||||
| DHEA 273.0 ng/dL | ||||||||||
| DHEAS ud | ||||||||||
| *** | 4.11 | TT 30.0 ng/dL | ||||||||
| DHEA 272.0ug/dL | ||||||||||
| 3 | 30 | 7.7 | ud | DHEAS 70.0ug/dL | ud | 1.4 | Secondary AI as part of pan- hypo-pituitarism | |||
| 8.7 | ud | FT 1.4 pg.mL | 1.7 | |||||||
| TT 14.0 ng/dL | ||||||||||
| DHEAS 56ug/dL | ||||||||||
| 4 | 46 | Crohn’s | 12.5 | ud | TT ud | ud | 1.4 | Secondary AI | ||
| Hypothyroid | FT ud | |||||||||
| DHEA 117.0ug/dL | ||||||||||
| DHEAS 13.0ug/dL | ||||||||||
1 POI primary ovarian insufficiency; 2 TPO thyroid peroxidase antibody; 3 TG thyroglobulin antibody; 4 DG deamidated gliadin antibody (IgA); 5 TG t-transglutaminase (igA) antibody; 6 ud undetectable; 7 SLE systemic lupus erythematosus treated with 7 mg prednisone p.o. o.d.; 8 HNA non-heredetary angioedema
*Only 14 of 29 women identified in the center’s research database with adrenal hypoandrogenemia have so far been investigated in follow up
** Not obtained since patients received long-term prednisone
*** Androgens and AMH level after supplementation with DHEA
Patient 1: Upon diagnosis with PAI, the patient initiated supplementation with hydrocortisone, and continued her supplementation with levothyroxine. Though this patient presented to our center with a diagnosis of POI, her FSH values did not support this diagnosis but a diagnosis of oPOI/POA
Patient 2: This patient consulted long-distance with our center after a spontaneous pregnancy loss in a spontaneously conceived pregnancy and after an IVF cycle suggestive of PCOS (29 oocytes), but with only 2 poor-quality embryos. After low androgens were noted, we recommended supplementation with DHEA 25 mg p.o., t.i.d. Androgen levels improved, as did her AMH, and the patient spontaneously conceived what was diagnosed as an ectopic pregnancy. We suspect this to represent a case of iatrogenic (tertiary) AI, secondary to prolonged prednisone supplementation
Patient 3: This patient presented to our center since us of a gestational carrier had been recommended to her elsewhere
Patient 4: This patient presented with primary infertility and Crohn’s disease, treated with Enbrel® (etanercept)