Background/Purpose: In the Southwestern United States, Coccidioidomycosis (cocci) or Valley fever is an endemic fungal infection. It typically presents as a self-limited pulmonary illness.  Patients with autoimmune diseases on disease-modifying antirheumatic drug (DMARD) or biologic response modifier (BRM) are at higher risk of more severe infection or infection disseminated outside the thoracic cavity. Currently, there are no management guidelines for cocci in such patients; however, retrospective data from the largest cohort of patients followed longitudinally suggests that retreatment of the rheumatic disease may be safe in some patients, and a management strategy based on cocci disease activity has been developed and implemented.

Methods: A retrospective chart review was performed and identified patients at two centers in Tucson, Arizona, who developed cocci on DMARD and/or BRM therapy. Patients were seen at least once between 2007 and 2014. Review emphasized the underlying rheumatic disease, manifestations of cocci, antifungal therapy, outcome, and management of BRM/DMARD therapy.

Results: 71 patients on DMARD and BRM therapy were identified. Among these patients, 19 were on BRM therapy, 16 were on DMARD and 36 were on combination therapy. Coccidioidomycosis was asymptomatic with positive serologies only in 19; 41 had pulmonary disease; while 10 were disseminated.  BRM and DMARD therapy was stopped in 37 while in 14, BRM was stopped but DMARD continued.  There was no change in immunosuppressive therapy in 20 patients.  52 patients were initially treated with antifungal therapy for 3 months or longer. Two of the patients with pulmonary illness and 12 patients with asymptomatic infection did not receive any antifungal therapy.

64 patients had follow up, out of which 50 patients continued BRM and/or DMARD therapy, and 22 received antifungal therapy. Four patients with disseminated disease restarted BRM therapy. Persistent positive serology and disseminated disease were the most common reasons for continuing antifungal therapy.  Conversion to negative serology was the most common reason for stopping antifungal therapy. Patients were followed for a median of 29 months (range 2-141).  There was one death from the group who was on DMARD and corticosteroid therapy. The deceased patient had disseminated coccidioidomycosis and was on antifungal treatment. There have been no deaths in the last three years. One patient with history of coccidioidal meningitis had relapse of disease, which was most likely related to his non-adherence with antifungal therapy.

Conclusion: Retreating or continuing DMARD and/or BRM therapy after cocci infection appears to be safe in some patients with close monitoring. If Cocci is asymptomatic, continuation of DMARD and/or BRM can be considered. In patients with pulmonary or disseminated infection, immunosuppressive therapy should be stopped and antifungal therapy should be continued until there is evidence of control of the infection. The management strategy implemented may be effective in guiding therapy.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/update-in-the-management-of-biologic-response-modifiers-and-disease-modifying-antirheumatic-drugs-following-coccidioidomycosis/