Endocrine Abstracts

Introduction: Autoimmune polyglandular syndrome (APS) is a rare disease, that is defined by the presence of two or more glandular insufficiencies caused by autoimmune mechanisms and that may be associated with other pathologies and immunological phenomena. It is characterized by the Presence of circulating organ specific antibodies and lymphocytic infiltration of the affected gland.

Case report: A 38-year-old female known to have autoimmune polyglandular syndrome type 2. 14 years ago, she wasdiagnosed with hypothyroidism and primary adrenal insufficiency on Eltroxin 100 mg and hostacortine 5 mg. 4 years ago, she developed type 2 diabeteson insulin mixtard 34 U before breakfast and 12 U before dinner. She had secondary amenorrhea for 4 years ago. Referred from emergency department with hyperkalemia.

On examination: The patient is fully conscious, Pulse: 90 beats/minute/BP: 190/100/RR: 16–20/minute, BMI: 23.55 kg/m². Normal head and neck, cardic, chest, abdominal examination.

Investigation showed: Leukocytosis (white blood cells: 14,100 cells/µl) with a neutrophil count of 84.9%), HB 13.1, normal coagulation profile, elevatedurea 79 mg/dl, elevated creatinine 1.15 mg/dl, low eGFR56 ml/mit, normal urine analysis, Albumin/Creatratio: 10 mg/mmol, normal sodium level. 135 mEq/l, hyperkaleamia (Potassium 9.7 mEq/l), normal calcium (9.2 mg/dl) normal phosphrous (4.9 mg/dl), hyperurceamia (8.3 mg/dl), arterial blood gases showing metabolic acidosis (pH: 7.31, PCo2: 33.2 mmHg, HCo3: 16.7 mmHg, Anion Gap: 12.6). Negative HCV, HBsAG. Renin >128 pg/ml(4–46)/Aldosterone 50.3 pg/ml (30–350). Thyroid profile normal (TSH: 2.82 mIU/l,FT4: 1.4 µg/dl, F T3: 91 ng/dl). Positive anti TPO. Post-menopausal FSH: 80.4 IU/l/post-menopausal LH: 84.3 IU/l. A 12-lead ECG revealed normal sinus rhythm with no hyperkalemic changes Abdominal sonar: Bilateral small sized kidneys. She sought medical advice and wasfound to have renal impairment (Creatinine 4 mg/dl then dropped to 1.4 mg/dl following hydration with IV fluids). Repeated admission with hyperkalemia resistant to anti hyperkalemic measures without any ECG changes and was placed on Kayexalate. Interstitial nephritis wassuspected however, our patient refused to do renal biopsy. Last creatinine done 1.5 mg/dl, eGFR 41 ml/mint. Here, we reported a case of APS-2 with an uncommon condition involved 3 endocrine organs (thyroid, adrenal, and ovarian) with interstitial nephritis.

Conclusion: Patient education, detailed evaluation, and long term follow up of patients and first-degree relatives for possible co-existence of diseases are the cornerstones for managing patients with APS.