Fatal septic shock due to disseminated coccidioidomycosis: a case series and review of the literature

Abstract

Background Coccidioidomycosis is a fungal infection endemic to the southwestern United States and regions of Latin America. Disseminated disease occurs in < 1% of cases. Septic shock is even rarer, with high mortality despite therapy. Case summary We describe two cases of coccidioidal septic shock. Both patients were older men of Filipino ancestry presenting with respiratory failure and vasopressor-dependent shock. Antifungal drugs were initiated after failure to improve with empiric antibiotics; in both, Coccidioides was isolated from respiratory cultures. Despite aggressive care, both patients ultimately died of their infections. We provide a review of the published literature on this topic. Conclusions Most of the 33 reported cases of coccidioidal septic shock occurred in men (88%) of non-white race and ethnicity (78%). The overall mortality rate was 76%. All survivors received amphotericin B as part of their treatment. Coccidioidomycosis-related septic shock is a rare disease with poor outcomes; delays in diagnosis and treatment are common. Improved diagnostic testing for coccidioidomycosis could enhance recognition of this disease in the future. Although data are limited, early treatment with amphotericin B in cases of coccidioidal septic shock may reduce mortality.

Fulminant Disseminated Coccidioidomycosis With Histoplasma Antigen Cross-Reactivity

“…Human-to-human transmission does not occur. Some 20,000 cases are reported annually in the United States with rates of infection on the rise, but fulminant disease occurs in <1% of all cases [ 4 ].…”

“…These symptoms often resolve spontaneously without antifungal therapy [ 1 , 2 ]. In fulminant disseminated disease, however, patients present with an advanced or accelerated course including involvement of extrapulmonary sites such as the central nervous system, skin, joints, and bone [ 4 ]. Our patient presented with nearly every radiographic lung abnormality that can be seen in pulmonary coccidioidomycosis, including consolidation, cavitation, cysts, bronchiectasis, and diffuse miliary nodules, the last being associated with hematogenous dissemination [ 7 ].…”

“…Some of these changes, such as bronchiectasis, were suggestive of pre-existing chronic lung disease or a more chronic infectious course, but in the setting of recently initiated corticosteroids for ITP, disease progression likely accelerated in the days prior to presentation. Diabetes mellitus may have also been a contributor [ 4 ].…”

“…Outcomes associated with disseminated coccidioidomycosis, especially with fulminant presentations such as patients with septic shock, are extremely poor with mortality rates >70%. [ 4 ] Mortality is likely related to a combination of delayed diagnosis and undertreatment with antifungal therapy. It is also likely that our patient was severely debilitated at baseline following his recent hospitalization, further contributing to his rapid deterioration.…”

“…Of these infections, most are self-limited and resolve without treatment [ 3 ]. However, more severe diseases with extrapulmonary involvement can occur in <1% of cases [ 4 ]. Here we present a case of fulminant, disseminated coccidioidomycosis in a patient recently started on oral corticosteroids.…”