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2012 | 7 | 3 | 358-361
Tytuł artykułu

Occam’s razor reveals a hidden Churg-Strauss syndrome

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A 28 year-old caucasian lady, with nine years of uncontrolled bronchial asthma, rhinosinusitis and mild upper limb paresthesia, came to our attention to be followed for coeliac disease (CD). She had a biopsy performed elsewhere which proved the diagnosis five years before. Since there was no clinical improvement on a strict gluten-free diet, we re-evaluated the slides of her duodenal biopsies and we found an overestimation of the duodenal lesions due to the wrong orientation of the specimens. Moreover, she had never had positive CD-related antibodies and she was negative for DQ2/DQ8 MHC Class II heterodimers. Months later, she referred she was suffering from diffuse joint pain, epistaxis and a substantial weight loss. A few days later she was hospitalized because of a sudden onset of dyspnea, peripheral edema and pleural effusion. Her echocardiogram showed global left ventricular hypokenesia with an ejection fraction of 24%. The patient was discharged with a diagnosis of dilated cardiomyopathy and NYHA Class II. After a large spectrum of haematological exams, the diagnosis of Churg Strauss Syndrome (CSS), a rare multisystemic small-vessel necrotizing vasculitis, was confirmed by the presence of four/five out of six diagnostic American College of Rheumatology classification criteria (Asthma, Eosinophilia >10%, Neuropathy, Non-fixed pulmonary infiltrates, Paranasal sinus abnormality and Biopsy containing a blood vessel with extravascular eosinophils). Our patient had been under-diagnosed by pulmonologist and by gastroenterologists although she presented the criteria required for CSS diagnosis. Our case report emphasizes that often seemingly unrelated symptoms can be caused by a single rare clinical complex.
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  • Department of Paediatrics, “Sapienza” University of Rome, Viale Regina Elena 324, 00161, Rome, Italy,
  • Department of Paediatrics, “Sapienza” University of Rome, Viale Regina Elena 324, 00161, Rome, Italy
  • Department of Paediatrics, “Sapienza” University of Rome, Viale Regina Elena 324, 00161, Rome, Italy
  • Department of Paediatrics, “Sapienza” University of Rome, Viale Regina Elena 324, 00161, Rome, Italy
  • Experimental Medicine and Pathology, “Sapienza” University of Rome, Viale Regina Elena 324, 00161, Rome, Italy
  • Department of Paediatrics, “Sapienza” University of Rome, Viale Regina Elena 324, 00161, Rome, Italy
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