Actinomyces in Chronic Granulomatous Disease: An Emerging and Unanticipated Pathogen

Abstract

Background Chronic granulomatous disease (CGD) is a rare inherited disease of the phagocyte NADPH oxidase system that causes defective production of toxic oxygen metabolites, impaired bacterial and fungal killing, and recurrent life-threatening infections, mostly by catalase-producing organisms. We report for the first time, to our knowledge, chronic infections with Actinomyces species in 10 patients with CGD. Actinomycosis is a chronic granulomatous condition that commonly manifests as cervicofacial, pulmonary, or abdominal disease, caused by slowly progressive infection with oral and gastrointestinal commensal Actinomyces species. Treatment of actinomycosis is usually simple in immunocompetent individuals, requiring long-term, high-dose intravenous penicillin, but is more complicated in those with CGD because of delayed diagnosis and an increased risk of chronic invasive or debilitating disease. Methods Actinomyces was identified by culture, staining, 16S ribosomal DNA polymerase chain reaction, and/ or a complement fixation test in 10 patients with CGD. Results All 10 patients presented with a history of fever and elevated inflammatory signs without evident focus. Diagnosis was delayed and clinical course severe and protracted despite high-dose intravenous antibiotic therapy and/or surgery. These results suggest an unrecognized and unanticipated susceptibility to weakly pathogenic Actinomyces species in patients with CGD because these are catalase-negative organisms previously thought to be nonpathogenic in CGD. Conclusions Actinomycosis should be vigorously sought and promptly treated in patients with CGD presenting with uncommon and prolonged clinical signs of infection. Actinomycosis is a catalase-negative infection important to consider in CGD.

Broncholithiasis Secondary to Pulmonary Actinomycosis

“…[3][4][5][6] Actinomyces are Gram-positive bacilli that are not acidfast, and produce branching, filamentous colonies that are round to oval in shape. 2,4,5,7 Actinomyces colonies produce sulfur granules that are yellowish in color. 4,5 Actinomyces species are considered normal flora of the human oropharynx, gastrointestinal tract, and urogenital tract, 5,7 but can cause infection by invading structures and organs through mucosal lesions.…”

“…2,4,5,7 Actinomyces colonies produce sulfur granules that are yellowish in color. 4,5 Actinomyces species are considered normal flora of the human oropharynx, gastrointestinal tract, and urogenital tract, 5,7 but can cause infection by invading structures and organs through mucosal lesions. 8,9 The incidence of actinomycosis is approximately 1 in 300,000 people.…”

“…8 Actinomycosis is confirmed by the isolation and culture of Actinomyces colonies and/or the histopathologic finding of sulfur granules. 4,7 Broncholithiasis is a condition in which calcified nodules or lymph nodes are identified within or migrating into a bronchial lumen. [10][11][12] The erosion of the calcified nodule into an airway is a result of continuous movement of the thoracic structures during active breathing or deglutition, 5,11,13 thereby causing partial or complete obstruction of the airway, potentially leading to an obstructive pneumonia.…”

Paravertebral Mushroom: Identification of a Novel Species of Phellinus as a Human Pathogen in Chronic Granulomatous Disease

“…In addition to the pathogens that are characteristically seen with CGD, there is an emerging list of microbes that have been identified as pathogens in these patients (7)(8)(9)(10)(11)(12). Phellinus sp.…”

Actinomycosis (Lumpy Jaw) in Capra Hircus Buck: A Case Report

“…To date a total of 42 different clinically important species of the microbe have been identified. Etiological agent is Gram-positive, non-spore forming, non-acid fast, facultative anaerobe with filamentous morphology (Reichenbach et al, 2009). The disease is likely to occur when there is breach in the continuity of mucosal layers followed by any injury, trauma or surgical course.…”