Periodic fever with pharyngitis, aphthous stomatitis and cervical adenitis (PFAPA syndrome) is a common cause of periodic fever in children and usually manifests as episodes of fever recurring with a clockwork periodicity. Although rare after adolescence, adult patients with PFAPA syndrome may present with a wider range of symptoms and may lack the clockwork periodicity of fever. A 24-year-old patient presented with a 4-year history of periodic fever with pharyngitis and cervical adenitis. She also complained of vomiting, fatigue and sporadically presented with aphthous stomatitis. During crises, laboratory evaluation showed a moderate elevation of inflammatory markers. Blood cultures and ANA titres were negative. Immunoglobulins and serum ferritin levels were normal. After other causes of periodic fever had been excluded, a diagnosis of PFAPA syndrome was made. LEARNING POINTS PFAPA syndrome is characterized by periodic fever accompanied by pharyngitis, aphthous stomatitis and cervical adenitis. It is a common cause of periodic fever in children but can also present in adults with a wider range of clinical manifestations. Establishing a firm diagnosis of PFAPA syndrome may avoid excessive work-up and potentially harmful treatment.
Senile systemic amyloidosis is caused by a non-mutated form of transthyretin with the heart being the major organ involved. This infiltrative cardiomyopathy usually presents as slowly progressive heart failure. An 82-year-old female patient was admitted for newly diagnosed heart failure. A year later she presented with decompensated heart failure and syncope. Inpatient work-up showed persistently elevated troponin and N-terminal-pro BNP levels, rapid progression to severe left ventricular concentric hypertrophy, and sinus pauses on the Holter. Cardiac MRI revealed diffuse late gadolinium enhancement in the left ventricle. The demonstration of amyloid protein with the clinical findings and complementary investigations allowed for the diagnosis of senile systemic amyloidosis.
Fungal infections caused by Paecilomyces species are rare. Although most cases of human infection occur in immunocompromised patients, this species is also being increasingly recognized as a pathogen in immunocompetent individuals. Paecilomyces variotii and P. lilacinus are the most common pathogens in humans. P. variotii infection usually manifests as peritonitis, pneumonia or endophthalmitis. We present the case of an 84-year-old immunocompromised patient with interstitial lung disease, who developed pulmonary mycetoma with isolation of P. variotii in bronchoalveolar lavage fluid culture. We found one similar case in the literature, but Paecilomyces species was not identified. Therefore, this is the first described case of pulmonary mycetoma caused by P. variotii.
Non-Hodgkin lymphomas are rare causes of primary lung neoplasms and most are B-cell in origin. Anaplastic large cell lymphoma is an exceedingly rare type of primary pulmonary lymphoma, with an aggressive clinical course.We present the case of an 85-year old male patient who attended our Emergency Department complaining of respiratory and constitutional symptoms, and who was found to have a bronchial mass causing subtotal atelectasis of the left lung. Histological examination showed an anaplastic large cell lymphoma and further investigation revealed that it was limited to the lung. To our knowledge, very few similar cases have been reported in the literature.LEARNING POINTSNon-Hodgkin lymphomas are rare causes of pulmonary lung neoplasms, with the majority of cases being marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue or diffuse large B-cell lymphoma.Anaplastic large cell lymphoma (ALCL) usually involves the lymph nodes, skin and soft tissue. It follows an aggressive clinical course and constitutional symptoms are frequent at presentation. Lung involvement may occur as a result of dissemination in up to 12% of cases. Primary ALCL of the lung is extremely rare.Anaplastic lymphoma kinase (ALK) expression is an important prognostic factor, with ALK+ ALCL patients experiencing better outcomes. Adverse prognostic factors also include advanced age, serum lactate dehydrogenase levels and early relapse after therapy.
We report the case of a 66-year-old man with dental infection who presented to our emergency department complaining of a 3-month medical history of chest pain and productive cough, in association with malaise, fever, weight loss and anaemia. His chest radiograph showed a nearly total opacification of the right hemithorax and chest ultrasound findings were suggestive of empyema, subsequently confirmed by a chest CT. The patient started appropriate treatment. A follow-up chest CT performed to rule out bronchopleural fistula revealed a large lung abscess. The patient had the final diagnosis of a giant lung abscess, which was initially thought to be an empyema because of the clinical and radiologic similarities with this entity. The initial misdiagnosis led to prompt percutaneous drainage of the lung abscess in addition to antibiotherapy and respiratory physiotherapy with a good final outcome, which suggests the efficacy of this approach in similar cases.
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