A complex case of disseminated histoplasmosis triggering hemophagocytic lymphohistiocytosis in a patient with lupus

Disseminated histoplasmosis is a rare yet serious fungal infection that primarily affects individuals with compromised immune systems. While it is widely known for its endemicity in the Midwest region of the USA, recent studies have indicated a noteworthy increase in sporadic cases, suggesting a widening of the endemic region for the pathology. This report describes a case of disseminated histoplasmosis in a 39-year-old female with a history of lupus, hypertension, anxiety, asthma, idiopathic edema, and fibromyalgia from a nonendemic region, who presented with cyclic fevers of unknown origin, peripheral edema, and oral sores. On admission, she was diagnosed with acute pyelonephritis and started on levofloxacin. She continued to develop worsening leukopenia and thrombocytopenia in addition to bone and joint pain. Bone marrow biopsy results were consistent with hemophagocytic lymphohistiocytosis (HLH) triggered by histoplasmosis confirmed by PCR. Despite an initial negative urine antigen test for Histoplasma, subsequent tests showed rising levels. The patient's clinical course was marked by a protracted hospital stay, multiple systems involvement, severe de-conditioning, drug side effects requiring adjustments in anti-fungal medications, and interdisciplinary care. The patient gradually improved and was discharged home with follow-ups. This study underscores the role of timely diagnosis of disseminated histoplasmosis in patients with underlying autoimmune diseases for favorable outcomes, thereby emphasizing the necessity of heightened clinical suspicion. By addressing the nuanced challenges that arise in managing multiple complications in the domain of disseminated histoplasmosis, advocates a comprehensive interdisciplinary approach to optimize patient care.