ABSTRACT
Hypereosinophilic syndrome is a rare condition characterized by eosinophilia associated with organ damage, most commonly affecting the skin, lung, gastrointestinal, cardiovascular and central nervous system. The idiopathic form is characterized by the absence of other conditions associated with hypereosinophilia such as allergies, infectious, hematological, immunological, endocrine or neoplasm diseases. The authors present a clinical case of a 70-year-old man with no relevant history, who went to the emergency department for neurological deficits and nonspecific chest pain. Laboratory tests revealed marked eosinophilia, elevation of cardiac enzymes with normal electrocardiogram. Computed tomography of the head showed multiple bilateral ischemic lesions. Upon further investigation for the cerebrovascular disease, transesophageal echocardiogram showed a thrombus at the aortic arch, as a probable embolic source. Despite anticoagulant therapy and corticosteroids, the patient's status deteriorated, with multiple successive ischemic strokes and worsening neurological deficits. After a thorough investigation, the diagnosis of idiopathic eosinophilia was established.
ABSTRACT
Granulomatosis with polyangiitis (Wegener's granulomatosis) is a systemic vasculitis that primarily affects small and medium vessels. Its manifestations are usually confined to the upper airway, lower airway and kidney. It can also affect other organs and systems, although this is unusual. We describe the case of a 67-year-old woman who presented with a tension pneumothorax due to rupture of a pulmonary cavity. This pulmonary cavity proved to be secondary to systemic disease which also caused a tumour in her kidney. Biopsy showed non-necrotizing granulomatosis, and even though antineutrophil cytoplasmic antibodies (ANCA) were negative, the diagnosis of granulomatosis with polyangiitis was made. LEARNING POINTS: Granulomatosis with polyangiitis (GPA) can be a challenging diagnosis when the initial manifestation is atypical, so a careful history and physical examination are needed to make the diagnosis.It is not uncommon for patients with multisystemic inflammatory disease to attend several different specialty clinics before the diagnosis is reached.GPA with negative ANCA is rare, and occurs more frequently in non-severe forms of the disease.