Infective Aortic Valve Endocarditis in a Patient With Mixed Connective Tissue Disease.

Mixed connective tissue disease (MCTD) is a rare autoimmune disorder that is characterized by overlapping clinical features of systemic lupus erythematosus (SLE), scleroderma, and myositis. Both SLE and mixed connective tissue disease patients are more prone to have acute endocarditis, and immunosuppression is a risk factor for recurrence of infective endocarditis. We present the case of a 53-year-old female with mixed connective tissue disease presenting with interstitial lung disease and precapillary pulmonary hypertension. The patient was chronically medicated with prednisolone, mycophenolate mofetil, and hydroxychloroquine. She was admitted for Enterococcus faecalis infective endocarditis and was treated with a four-week course of ceftriaxone and ampicillin. Immunosuppressive chronic medication was maintained due to severe lung involvement. One month later, the patient was re-admitted due to respiratory infection with identification of influenza B virus. However, fever persisted for over one week, and subsequent relapse of the E. faecalis infective endocarditis was found. The diagnosis was made based on blood cultures and a transoesophageal echocardiogram. No other focus of infection was identified. She completed a six-week course of vancomycin and gentamicin and underwent cardiac surgery with success. This case highlights the difficulty of the management of immunosuppressed patients in the presence of serious infections.

Bone Marrow Granulomatosis in Acute Q Fever.

Reported cases of Q fever in people living in urban areas after occasional contact with farm animals or infected pets such as dogs and cats have been increasing. The diagnosis of Q fever is usually laborious due to unspecific and variable clinical manifestations. The most common clinical presentation is an influenza-like illness with varying degrees of pneumonia and hepatitis. Acute hepatitis is more frequent than pneumonia in countries where the disease is endemic, such as in Portugal. We report a case of acute Q fever with hepatic and bone marrow involvement presented as fever of unknown origin (FUO) in a 56-year-old sportive hunter man. Typical fibrin ring granulomas (doughnut granulomas) were found in the bone marrow biopsy and were essential for the diagnosis. Bone marrow involvement is considered a rare manifestation of Q fever. Coxiella infection activates a granulomatous inflammatory response that can lead to persistent immune cell activation. Doughnut granulomas are not pathognomonic but they are highly specific for the diagnosis of Q fever.

Native Valve Aspergillus Endocarditis in a Non-Neutropenic Immunocompromised Patient on Anti-TNF α Blockers Therapy.

Invasive aspergillosis is a rare opportunistic infection mainly occurring in patients with a well-established risk such as neutropenia or conditions that lead to chronically impaired cellular immune responses. Systemic corticosteroids are a well-known risk factor for fungal infections. Recently, reports of invasive aspergillosis in patients treated with monoclonal biologic agents, such as tumor necrosis factor-alpha inhibitors, have been increasing. We present the case of a 47-year-old female patient with seronegative spondyloarthropathy treated with infliximab and corticosteroids. The patient presented classical symptoms of an acute lower respiratory infection, and she was treated with a ß-lactam antibiotic. Infliximab administration was deferred until nine days after clinical recovery. Fourteen days after drug administration, she was admitted with a symptomatic subcortical hematoma in the left parietal region. There was a rapid neurological recovery, and there were no risk factors for haemorrhagic stroke detected. The chest X-ray revealed an oval mass with an air crescent sign, and the CT scan was suggestive of aspergilloma. Bronchoalveolar lavage cytology identified Aspergillus spp. Voriconazole was initiated and, after one month of treatment, the patient was readmitted with a left facial palsy associated with hemiparesis and dysarthria. Laboratory evaluation showed leukocytosis and elevated C-reactive protein. A severe right middle cerebral artery stroke was present on the brain CT scan. Transesophageal echocardiogram revealed large mitral valve vegetation, and the diagnosis of Aspergillus endocarditis with cerebral embolization was made. Fungal infections are challenging due to the diagnosis infrequency and paucisymptomatic natural history. Despite being crucial in the treatment of autoimmune diseases, immunosuppressive drugs increase the risk of fungal infection. It is extremely important to consider Aspergillus infection in immunosuppressed patients, and the need for prophylaxis in non-neutropenic patients with risk factors should be clarified.