Fungal pericarditis is a rare disease but its incidence has risen in parallel with the global increase in invasive fungal infections. This systematic review analyzes data from previously reported cases of fungal pericarditis to provide an improved understanding of the etiology, clinical presentation, management, and outcomes of this rare disease. We reviewed Medline and Scopus databases from 1 January 1990 to 29 January 2024 for case reports that documented the isolation of a fungal pathogen from pericardial fluid or tissue. Of the 2330 articles screened, 101 cases met the inclusion criteria. Patients with fungal pericarditis and the involvement of at least one other organ—usually the lungs, brain, or kidney—had worse outcomes than patients with isolated pericardial disease. Immunosuppression was reported in 50% of cases and was associated with worse outcomes in adults. Patients who presented with chest pain, received adequate empiric antifungal therapy, and underwent pericardiocentesis and pericardiectomy had improved survival. The most common isolated pathogens were Candida spp., followed by Aspergillus spp. and Mucor spp., with the latter two linked to worse outcomes. Only 35% of patients received empiric antifungal medications before the causative pathogen was identified, and mortality was associated with a delay in appropriate therapy. Immunosuppression, disseminated disease, and presence of shock/multiorgan failure were additional risk factors associated with death. Fungal pericarditis carries a mortality rate of up to 50%, with nearly half of patients being immunocompromised. Clinicians frequently do not consider fungal pericarditis in the differential diagnoses, which leads to delays in treatment and poorer outcomes. Further prospective multicenter studies are urgently needed to better understand the epidemiology, improve diagnostic testing and management, and decrease unacceptably high mortality in patients with fungal pericarditis.
Recurrent Intestinal Angioedema with Normal C1-Inhibitor: A Case Report
Background and Objectives: Angioedema is a non-pitting edema of the submucosal layer which can be acquired or inherited and usually presents with hives. Intestinal angioedema is rare and can mimic other acute gastrointestinal disorders. It is typically associated with a lack or dysfunction of C1-inhibitor, with a small number of cases having normal C1-inhibitor function. We present a rare case of chronic recurrent intestinal angioedema in a patient with normal C1-inhibitor function who did not respond to icatibant therapy. Case presentation: A 56-year-old woman presented with 3 days of abdominal pain, nausea, vomiting, and diarrhea. She denied a history of allergies and reported a 30-year history of similar episodes requiring hospitalization. Initial evaluation demonstrated normal C4 and C1 esterase inhibitor function with negative gastrointestinal bacterial and viral panel. A CT of the abdomen and pelvis demonstrated acute diffuse bowel thickening and prominent mesenteric lymph nodes. MRI demonstrated inflammation of the small and large bowel. EGD and colonoscopy findings were normal. She was diagnosed with intestinal angioedema and started on icatibant without significant improvement. Her symptoms resolved after 3 days of supportive therapy and resolution of inflammation was noted on imaging. She was discharged home with allergy and immunology follow-up. Conclusions: Intestinal angioedema is under-recognized and presentation can overlap with other pathologies of the GI tract. Extensive work up is needed during the first episode of an attack and complement levels should be checked in all patients. Appropriate classification is important as it dictates therapy. However, ambiguous cases like ours sometimes cannot be classified into any specific category.
Suppurative portal vein thrombosis (pylephlebitis) is an uncommon condition usually associated with an intra-abdominal infection or inflammatory process. In this study, we aimed to synthesize data on previously published cases according to the PRISMA guidelines. A total of 103 patients were included. Patients were more commonly male (71.8%) and had a mean age of 49 years. The most common infection associated with pylephlebitis was diverticulitis (n = 29, 28.2%), and Escherichia coli was the most isolated pathogen (n = 21, 20.4%). Blood cultures were positive in 64 cases (62.1%). The most common site of thrombosis was the main portal vein (PV) in 59 patients (57.3%), followed by the superior mesenteric vein (SMV) in 40 patients (38.8%) and the right branch of the PV in 30 patients (29.1%). Sepsis developed in 60 patients (58.3%). The mortality rate in our review was 8.7%, and independent risk factors for mortality were the presence of pertinent comorbidities (OR 5.5, p = 0.02), positive blood cultures (OR 2.2, p = 0.02), and sepsis (OR 17.2, p = 0.049).
Primary Lymphocutaneous Nocardia brasiliensis in an Immunocompetent Host: Case Report and Literature Review
Nocardia spp. is a Gram-positive, partially acid-fast aerobic bacterium usually associated with infection in immunocompromised people. The most common sites of infection are the skin, lungs, and the brain, however disease can disseminate and affect every organ. Clinical manifestations of cutaneous disease are varied and frequently misdiagnosed. We present a case of an immunocompetent 66-year-old man who sustained a left finger injury while gardening. He was misdiagnosed on several occasions and treated with inappropriate antibiotics against Streptococcus spp. and Staphylococcus spp. When infection spread cutaneously, sporotrichoid (lymphocutaneous) nocardiosis was suspected and the patient was started on appropriate therapy with Bactrim which resulted in a cure. We also summarize the literature on lymphocutaneous infection by Nocardia brasiliensis. By reporting this case, we want to raise awareness among clinicians about unusual causes of cellulitis, the differential diagnosis of lymphocutaneous infection and the importance of obtaining a detailed exposure history to assist in the prompt diagnosis of nocardiosis.