Disseminated Cryptococcosis Causing Primary Adrenal Insufficiency in a Patient with Idiopathic T-Cell Lymphopenia

Presentation Number: SAT-0757
Date of Presentation: June 21st, 2014

Lauren Brian Hawkins*1 and Cynthia Anne Burns2
1Wake Forest University School of, Winston Salem, NC, 2Wake Forest Univ Hlth Sci, Winston Salem, NC

Abstract

Background: Disseminated Cryptococcosis typically affects immunocompromised patients, most often affecting the CNS and lungs. Very rarely cryptococcus causes adrenal insufficiency. Adrenal disease without evidence of CNS and lung disease is rare.

Clinical Case:We present an atypical case of primary AI secondary to cryptococcal infection without evidence of CNS involvement.

The patient was admitted to the hospital with hypotension, weight loss, and N/V. A 250mcg cosyntropin stimulation test confirmed primary AI: baseline cortisol 1.4 mcg/dL (N 5.5-20.0 mcg/dL), cortisol at 30 minutes 1.8 mcg/dL, cortisol at 60 minutes 1.7 mcg/dL; ACTH 726 pg/mL (N 6.0-50.0 pg/mL). Treatment with hydrocortisone and fludrocortisone was begun.  Antibodies for 21-hydroxylase and adrenal tissue were negative.

Abdominal CT revealed symmetric, diffusely enlarged and thickened adrenal glands.  He was referred to ID for workup of adrenal infection. Serum cryptococcal Ag titer was positive at 1:64.  Testing for HIV was negative.  LP showed normal CSF protein (69 mg/dL, N 15-60 mg/dL), WBC (1/mm3, N 0-5/mm3), and glucose (44 mg/dL, N 50-75 mg/dL). CSF cryptococcal Ag was negative. Blood and CSF fungal cultures were negative. Chest CT showed a focus of lobular nodularity with surrounding groundglass attenuation in the RLL and numerous sub-4mm nodules in the right lung.  Induction therapy with liposomal amphotericin and flucytosine was given. He is currently taking fluconazole 200mg daily for maintenance therapy.

A low CD4 count at 150 (N 310-3,110) was found.  Immunoglobulins were also found to be low, likely secondary to poor T-cell stimulation: IgG 523 mg/dL (N 635-1741 mg/dL), IgA 30 mg/dL (N 70-350 mg/dL), IgM 26 mg/dL (N 70-210 mg/dL). He was referred to Immunology for further workup. He was prescribed weekly SC IG. No cause for his immunodeficiency has been found.

A follow up contrasted abdominal CT seven months after diagnosis showed unchanged adrenal enlargement. There was no contrast enhancement, suggesting no residual functioning tissue. Serum cryptococcal titer most recently is stable at 1:32 after eleven months of treatment

He is followed by Endocrinology, Infectious Disease, and Immunology. He continues hydrocortisone, fludrocortisone, fluconazole, weekly SC immunoglobulin, and PCP and HSV prophylaxis. The fluconazole will be continued indefinitely given his immune deficiency.

Conclusion: This patient’s case is atypical in that Cryptococcus rarely causes primary adrenal insufficiency, and when it does, it is typically associated with cryptococcal meningitis. We could only find three prior case reports of patients with adrenal insufficiency secondary to cryptococcosis without meningoencephalitis. This patient’s case is unique in that his diagnosis of cryptococcosis led to the finding of idiopathic T-cell deficiency and hypogammaglobulinemia, which up to this point had gone undiagnosed.

 

Nothing to Disclose: LBH, CAB