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2012 | 7 | 3 | 358-361
Tytuł artykułu

Occam’s razor reveals a hidden Churg-Strauss syndrome

Treść / Zawartość
Warianty tytułu
Języki publikacji
EN
Abstrakty
EN
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.
Słowa kluczowe
Wydawca
Czasopismo
Rocznik
Tom
7
Numer
3
Strony
358-361
Opis fizyczny
Daty
wydano
2012-06-01
online
2012-03-29
Twórcy
  • 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
Bibliografia
  • [1] Watts RA, Lane S, Scott DG. What is known about the epidemiology of the vasculitides? Best Pract Res Clin Rheumatol 2005; 19:191–207 http://dx.doi.org/10.1016/j.berh.2004.11.006[Crossref]
  • [2] Mohammad AJ, Jacobsson LT, Mahr AD, et al. Prevalence of Wegener’s granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg-Strauss syndrome within a defined population in southern Sweden. Rheumatology (Oxford) 2007; 46:1329–1337 http://dx.doi.org/10.1093/rheumatology/kem107[Crossref]
  • [3] Pagnoux C, Guilpain P, Guillevin L. Churg-Strauss syndrome. Curr Opin Rheumatol 2007; 19:25–32 http://dx.doi.org/10.1097/BOR.0b013e3280119854[Crossref]
  • [4] Sinico RA, Di Toma L, Maggiore U, et al. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum 2005; 52:2926–2935 http://dx.doi.org/10.1002/art.21250[Crossref]
  • [5] Megiorni F, Mora B, Bonamico M, et al. HLA-DQ and risk gradient for celiac disease. Hum Immunol. 2009;70:55–59 http://dx.doi.org/10.1016/j.humimm.2008.10.018[Crossref]
  • [6] Klion AD, Bochner BS, Gleich GJ, et al. Approaches to the treatment of hypereosinophilic syndromes: a workshop summary report. J Allergy Clin Immunol 2006; 117:1292–1302 http://dx.doi.org/10.1016/j.jaci.2006.02.042[Crossref]
  • [7] Hellmich B, Holl-Ulrich K, Merz H, Gross WL. Hypereosinophilic syndrome and Churg-Strauss syndrome: is it clinically relevant to differentiate these syndromes? [in German]. Internist (Berl) 2008; 49:286–296 http://dx.doi.org/10.1007/s00108-007-2009-4[WoS][Crossref]
  • [8] Masi AT, Hunder GG, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss Syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 1990;33:1094–1100 http://dx.doi.org/10.1002/art.1780330806[Crossref]
  • [9] Singh R, Singh D, Abdou N. Churg-Strauss syndrome presenting as acute abdomen: are gastrointestinal manifestations an indicator of poor prognosis? Int J Rheum Dis 2009;12:161–165 http://dx.doi.org/10.1111/j.1756-185X.2009.01399.x[Crossref]
  • [10] Cojocaru M, Cojocaru IM, Silosi I et al. Gastrointestinal manifestations in systemic autoimmune diseases. Maedica. 2011;6:45–51
  • [11] Guilpain P, Auclair JF, Tamby MC, et al. Serum eosinophil cationic protein: a marker of disease activity in Churg-Strauss syndrome. Ann N Y Acad Sci 2007; 1107:392–399 http://dx.doi.org/10.1196/annals.1381.041[Crossref]
  • [12] Tsukadaira A, Okubo Y, Kitano K, et al. Eosinophil active cytokines and surface analysis of eosinophils in Churg-Strauss syndrome. Allergy Asthma Proc 1999; 20:39–44 http://dx.doi.org/10.2500/108854199778681486[Crossref]
  • [13] Polzer K, Karonitsch T, Neumann T, et al. Eotaxin-3 is involved in Churg-Strauss syndrome: a serum marker closely correlating with disease activity. Rheumatology (Oxford) 2008; 47:804–808 http://dx.doi.org/10.1093/rheumatology/ken033[Crossref][WoS]
  • [14] Ribi C, Cohen P, Pagnoux C, et al. Treatment of Churg-Strauss syndrome without poor-prognosis factors: a multicenter, prospective, randomized, open-label study of seventy-two patients. Arthritis Rheum 2008; 58:586–594 http://dx.doi.org/10.1002/art.23198[Crossref]
Typ dokumentu
Bibliografia
Identyfikatory
Identyfikator YADDA
bwmeta1.element.-psjd-doi-10_2478_s11536-011-0163-5
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