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

Summary

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 anti‐fungal therapy.

Design

Single‐center retrospective hospital‐based study of 40 consecutive adults with AH [39 males; age (mean + SD) 53±11 years], conducted between 2006‐2018. 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 anti‐fungal therapy in 21 patients.

Results

Seventy three percent 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 anti‐fungal therapy, 25 (86%) were in remission at last follow‐up. Overall, 8 (20%) patients died: 3 had a sudden death, 4 had severe histoplasmosis and 1 died due to adrenal crisis. No patient with PAI became eucortisolemic on re‐testing after one‐year of anti‐fungal 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 anti‐fungal therapy, overall mortality was significant. Cortisol insufficiency did not normalize despite treatment.

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