Strongyloidiasis related hyper-infection syndrome; rare disease with catastrophic outcome.
Farooq MA, Dabbagh M, and Uduman J. Strongyloidiasis related hyper-infection syndrome; rare disease with catastrophic outcome. Am J Respir Crit Care Med 2018; 197.
Am J Respir Crit Care Med
Introduction:Strongyloidiasis is a nematode infection caused by Strongyloides stercoralis that can result in severe systemic hyperinfection syndrome characterized by massive dissemination of filariform larvae, which can result in multi-organ failure and death in an immunocompromised individual. This condition is endemic in tropical regions of the world. In the United States, it is commonly found in immigrants, military personnel or travelers to endemic areas. Our patient illustrates the subtle features and risk factors one must be aware of to promptly identify and treat Strongyloidiasis to avoid a catastrophic clinical outcome. Case Presentation: A 64-year-old male on oral steroids for a recent diagnosis of Immunoglobulin-G4 (Ig-G4) related aortitis four months ago, was admitted with recurrent constipation, diffuse abdominal pain and distension. Of note, he recently emigrated from South-East Asia. He had multiple hospital admissions recently due to similar complaints, treated with antibiotics for presumed abdominal infection without complete resolution of the symptoms. Computed Tomography (CT) scan of abdomen on admission showed bowel thickening consistent with non-specific enteritis and partial small bowel obstruction (SBO). He was deemed not be a surgical candidate due to initial resolution of symptoms with supportive therapy. Initial microbiology including, respiratory cultures, blood and urine cultures were all consistently negative. Despite transient improvement, he had recurrence of abdominal symptoms and developed acute respiratory failure and shock. He was treated with empiric antibiotics for septic shock. Chest x-ray suggested pneumonia, follow up CT-abdomen showed unresolved enteritis with SBO. Blood and urine culture remained negative, however, respiratory and stool cultures showed many larvae, identified as Strongyloides stercoralis. Patient was started on Ivermectin and Albendazole for strongyloides hyperinfection along with broad spectrum antibiotics due to risk of gram negative bacterial translocation from the gut. Patient's condition continued to deteriorate with progressive multi-organ failure that resulted in death. Discussion: Strongyloidiasis related hyperinfection syndrome is a life threatening condition with increased likelihood of developing in immunosuppressed state like recent use of steroids, hematopoietic malignancy or stem cell transplant. Even short courses of corticosteroids can lead to overwhelming hyperinfection syndrome. Our patient's onset of abdominal symptoms correlates well with the initiation steroids, which is a well-known risk factor for the development of hyperinfection syndrome. It is crucial for physicians to recognize this condition in non-endemic areas like the United States since delay in therapy can have fatal results. It is also imperative to screen high-risk patients for Strongyloides prior to initiation of immunosuppressive therapy.