Clinical outcomes and cortical reserve in adrenal histoplasmosis—A retrospective follow‐up study of 40 patients

Abstract

Objective: Detailed studies of Addison's disease resulting from disseminated adrenal histoplasmosis (AH) are not available. We describe the presentation and prognosis of AH and cortisol status before and after antifungal therapy.Design: Single-centre retrospective hospital-based study of 40 consecutive adults with AH [39 males; age (mean ± SD) 53 ± 11 years] was conducted between 2006 and 2018. The median duration of follow-up was 2.5 years (range 0.2-12 years). Patients and methods: AH was diagnosed by bilateral adrenal enlargement on CT scan and presence of Histoplasma by histology and/or culture of biopsied adrenal tissue. All patients received oral itraconazole and, if required, amphotericin B as per guidelines. ACTH-stimulated serum cortisol (normal > 500 nmol/L) was measured in 38 patients at diagnosis and re-tested after one year of antifungal therapy in 21 patients. Results: Seventy-three per cent of patients had primary adrenal insufficiency (PAI) and one-third had an adrenal crisis at presentation. HIV antibody was negative in all patients. Of the 29 patients who completed antifungal therapy, 25 (86%) were in remission at last follow-up. Overall, 8 (20%) patients died: three had a sudden death, four had severe histoplasmosis and one died due to adrenal crisis. No patient with PAI became eucortisolemic on re-testing after one year of antifungal therapy. Of the eight patients with normal cortisol at diagnosis, two developed adrenal insufficiency on follow-up.Conclusion: All patients with AH tested negative for HIV antibody. While patients achieved a high rate of clinical remission after antifungal therapy, overall mortality was significant. Cortisol insufficiency did not normalize despite treatment. K E Y W O R D SAddison's disease, adrenal insufficiency, cortisol, histoplasmosis | 535 SINGH et al.

The Review of Histoplasmosis Endemicity and Current Status in Asia

“…The cases from Bangladesh were all adult males, with 15% (four patients) who had acquired immunodeficiency syndrome (AIDS). The predominance of male population with histoplasmosis was also found in many case series [42,50,59]. The most common type of histoplasmosis was disseminated histoplasmosis.…”

“…The growing population of diabetes might contribute to the common findings of diabetes as a risk factor in histoplasmosis. Diabetes was commonly found as a predisposing factor in the mortality of adrenal histoplasmosis [50].…”

“…Adrenal histoplasmosis (44%, n = 54) was the most common manifestation of histoplasmosis among 124 histoplasmosis cases with HIV negative. A recent study reported an adequate response of antifungal therapy in adrenal histoplasmosis with the rate of 86% with a median duration of follow-up of 2.5 years [50]. However, the mortality is high (20%, 8 out of 29 patients) even among patients with completed therapy.…”

Adrenal Insufficiency Caused by Paracoccidioidomycosis: Three Case Reports and Review

“…In an autopsy series, the adrenal glands were affected in nearly 80% of patients with progressive disseminated histoplasmosis (PDH), which is more common in immunosuppressed individuals (11). The causal agent, Histoplasma capsulatum, leads to PAI more than any other fungal infection (12).…”

“…The causal agent, Histoplasma capsulatum, leads to PAI more than any other fungal infection (12). The combination between PDH and PAI usually results in a high mortality rate (11,12).…”