Pulmonary cryptococcosis in childhood systemic lupus erythematosus and Sjögren syndrome overlap: a rare opportunistic infection

Abstract

Meningitis is the main manifestation of cryptococcosis in adult systemic lupus erythematosus (SLE) patients, and other organs and systems, such as the lungs, are rarely affected in this fungal infection. To our knowledge, no case of pulmonary cryptococcosis has been described in the pediatric lupus population. Therefore, we report herein one patient with childhood SLE (C-SLE) and Sjögren's syndrome overlap that presented encapsulated Cryptococcus yeast cells in lung tissue. A 14-year-old girl was diagnosed with C-SLE. At the age of 16 years and 5 months, she presented with fever, cough and dyspnea, without headache, vomiting, and also without signs of meningeal irritation or other clinical manifestations. She was being treated with mycophenolate mofetil, hydroxychloroquine and prednisone. Chest radiography and chest computer tomography showed a single nodule in the left posterior apex and three nodular lesions in the left hemithorax respectively. Bronchoalveolar lavage and transbronchial biopsy were normal and without isolation of bacteria or fungi. Voriconazole was empirically introduced for 21 days. Fifteen days after the first biopsy, she underwent open thoracotomy with surgical left lung biopsy and was diagnosed with pulmonary cryptococcosis. Voriconazole was replaced with oral fluconazole and this antifungal therapy was maintained with improvement of clinical manifestations and without marked alteration of radiological images. In conclusion, we report the first case of pulmonary cryptococcosis in Sjögren's and C-SLE patient with a satisfactory clinical response to antifungal therapy. Fungal infections should be excluded in the presence of lung nodules and etiological identification is required for proper treatment.

A Multicenter Study of Invasive Fungal Infections in Patients with Childhood-onset Systemic Lupus Erythematosus

“…However, studies solely evaluating invasive fungal infections (IFI) in patients with cSLE, such as candidiasis, aspergillosis, cryptococcosis, and histoplasmosis, are limited to case reports and case series 7,9,10,11,12 without any systematic evaluation of the associated risk factors and outcomes in the cSLE population.…”

Mycophenolate mofetil increases susceptibility to opportunistic fungal infection independent of lymphocytes

“…There are specific case reports that identify cryptococcal infection in patients treated with mycophenolate (Kluger et al, 2009;Marques et al, 2013), and the Transplant-Associated Infection Surveillance Network (a consortium of 23 US transplant centres) identified a 12-month cumulative incidence of invasive fungal infection (IFI) as 3.1%, with cryptococcosis the cause of almost 10% of IFIs (Pappas et al, 2010).…”

Rapid Fatal Outcome of Cryptococcal Meningoencephalitis in a Non-HIV Immunocompromised Patient with a Low Fluconazole Susceptibility Isolate: A Case Report from Madagascar

“…However, cryptococcosis does not necessarily come to mind in an apparently immunocompetent individual. Depending on the site of invasion of the pathogen, a disseminated cryptococcal infection can be manifested in various clinical forms that include meningoencephalitis, pulmonary disease, skin disease, osteomyelitis, peritonitis, or comorbidities [4–9]. In Madagascar, little is known on the epidemiology or the clinical presentations of cryptococcosis.…”