Pôster:

The eosinophilic granulomatosis with polyangiitis is a systemic necrotizing vasculitis of small and medium vessels, primary and ANCA associated, which manifests with vasculitis, asthma and eosinophilia. The following report presents the diagnostic challenged posed by a patient with a typical clinical condition, however foreign to the classical epidemiology of the disease.

Case report

R.M.A., 28 years-old, Caucasian, admitted to the emergency room reporting daily fever, dyspnea to minimal efforts, coughing, hemoptysis and unquantified weight loss. He referred recent use of antibiotics, for a possibly infectious respiratory condition, and history of asthma since childhood, exacerbated on the last 2 months. On the exam, he presented with fever, tachycardia, tachypnea, crepitant rales on pulmonary bases, signs of hypoxemia, without adenomegaly. Thoracic radiography demonstrated areas of hypotransparency on both hemithoraces, the laboratory tests indicated leukocytosis with left shift and mild anemia and the echocardiogram showed no alterations. Piperacilin/Tazobactam and Claritromic were initiated, without clinical response. The following attempt instituted empirical RHZE scheme and corticotheraphy, this time with improvement of the symptoms. The patient was discharged and referred to the tuberculosis program. On the same week, he returned to the hospital with an aggravation of his initial respiratory condition, requiring admission to the ICU. He had developed new skin lesions and paresthesia on his legs and feet. The hemogram pointed leukocytosis with eosinophilia and the Computerized Tomography (CT) of the facial sinuses showed nasal polyps. For diagnostic elucidation, a skin biopsy was made – identifying leukocytoclastic vasculitis, and the p-ANCA was requested – its result was positive. Pulse theraphy with Methylprednisolone was initiated and followed with maintenance treatment with prednisone, obtaining a discreet improvement initially, but with significant clinical deterioration later on. The patient was submitted to a lung biopsy (Image 1), which indicated small vessels vasculitis with intense eosinophilic infiltrate, closing the diagnosis of eosinophilic granulomatosis with polyangiitis. Induction theraphy with cyclophosphamide was established, along with corticotheraphy – with gradual weaning. The patient evolved well, showing clinical, radiological and laboratory improvement, starting maintenance treatment with azathioprine.

Conclusion

Diagnosis of eosinophilic granulomatosis polyangiitis on this patient isn’t simple, especially considering the epidemiological dissonance, for it isn’t only a rare disease, but it classically demonstrates a preference for individuals of 60 years-of-age and over. Nevertheless, gathering the history of atopy, distinctive on the disease, with the respiratory symptomatology and vasculitis-suggestive skin lesions, as well as the eosinophilia and histological finding compatible with the disease, strongly suggest the diagnosis.