Adrenal Paracoccidioidomycosis: An Infrequent Etiology of Primary Adrenal Insufficiency

Friday, May 13, 2022

10:10 AM – 10:25 AM

Location: Station 10, Sapphire West Foyer

Submitter(s)

MF
María Carolina Fragozo-Ramos, MD
Endocrinology and Metabolism Fellow
Universidad de Antioquia
Medellin, Colombia

Primary Author(s)

FM
Fragozo-Ramos MC

Co-Author(s)

GL
Gómez-Galvis LV
GV
Gómez-Sierra V
RA
Román-González Alejandro, MD
Professor
Universidad de Antioquia-Hospital Universitario San Vicente Fundacion

Introduction : Primary adrenal insufficiency (PAI) is an uncommon entity. In developed countries the main cause is autoimmunity. In low- and medium-income countries infectious agents are the principal etiology. Paracoccidiomycosis (PCM) as an etiological agent of PAI is infrequent and is considered a fungus exclusive of South America. We describe a series of cases of four patients who presented PAI secondary to PCM.

Case Description : Case 1: A 54-year-old man presented to the hospital with abdominal pain and was diagnosed with acute appendicitis and underwent surgical management. On second post-operative day the patient presented refractory shock. Adrenal insufficiency was suspected. Initial laboratory workup showed sodium 134 mg/dL, potassium 5.4 mg/dL, cortisol at 8 am of 0.8 ug/dL (6–23 ug/dL) and adrenocorticotropic hormone (ACTH) of 383.8 pg/mL (6–76 pg/mL). Abdominal computed tomography (CT) showed an increase in the size of adrenal glands. A CT guided adrenal biopsy was performed and the presence of Paracoccidioides braziliensis was found. Case 2: 61-year-old man with long history of general malaise and unprovoked, unquantified weight loss. He underwent multiple blood tests confirming PAI with adrenal enlargement. An adrenal biopsy was done which found fungal structures with multiple budding characteristics of Paracoccidioides. Case 3: 62-year-old man presented with unprovoked weight loss of 13 kg, diaphoresis, nausea and subjective fever. The laboratories reported; sodium of 110 mg/dL, potassium of 5.8 mg/dL, cortisol at 8 am of 0.74 ug/dL (6–23 ug/dL) and ACTH ˃1250 pg/mL (6–76 pg/mL). Abdominal CT showed an increase in the size of adrenal glands. Left adrenalectomy was performed and the histopathology study showed PCM. Case 4: 68 years old male with history of asthenia, adynamic, and unprovoked weight loss of 10 kg. The laboratory work up showed potassium of 8.7 mg/dL and sodium of 121 mg/dL, cortisol at 08 00 am 0.4 ug/dL (6–23 ug/dL), ACTH of 1266 pg/mL (6–76 pg/mL) and abdomen CT showed diffuse thickening of the left adrenal gland and right adrenal mass of 50x25mm. Right adrenalectomy was performed and the histopathology study reported the presence of mycotic structures with multiple budding, diagnosing PCM. All subjects received specific antifungal treatment with adequate glucorticoid and mineralocorticoid replacement.

Discussion : We presents four cases of adrenal PCM. All cases show the importance to investigate PAI in subjects with compatible clinical findings. Based in our data, the presence of adrenal enlargement or masses in patients with PAI is suggestive of infectious etiology. The fungal infections should be considered among the diagnostic hypotheses during the etiological investigation of PAI. An invasive procedure is needed in most cases to diagnosis the putative agent.