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Unusual presentations of Churg Strauss syndrome in paediatrics: a tale of 2 masses from one centre experience
Pediatric Rheumatology volume 9, Article number: P96 (2011)
Background
To highlight unusual presentations of Churg Strauss Syndrome (CSS) in children.
Aim
Raising clinical awareness of atypical presentations.
Methods
Case note review and literature search.
Results
Case 1
A 15 year old girl with worsening asthma and rhinitis presented with a persistent 10x6 cm breast lump 6 months after starting Montelukast. Chest X-rays and CT scan were normal, maxillary sinuses congested, with absolute eosinophilia of 2.4 x 109/l, ESR normal, no proteinuria). On needle biopsy, dilated ducts contained necrotic eosinophilic debris, intense infiltrate of eosinophils and plasma cells without granulomatous infiltration or vasculitis. Despite withdrawing Montelukast the mass persisted but treatment with steroids was successful.
Case 2
A 14 year old girl presented with worsening asthma associated with malaise, weight loss, an intermittent vasculitic rash and abdominal pain. She had a large right ventricular mass with pericardial effusion and endocardial impairment, pulmonary infiltrates, microscopic haematuria, vasculitic changes on cerebral MRI, a raised eosinophil count (6.16x109), anaemia, ESR 114 and positive pANCA (non PR3 / MPO). A good clinical response to treatment with steroids, cyclophosphamide (IV) and immunoglobulin is currently maintained on MMF and low dose steroid with anticoagulation (warfarin). Her cardiac mass has not regressed, but remained static, most likely representing chronic endocardial fibrosis.
Conclusion
These cases illustrate atypical presentations of CSS in children. Eosinophilic mastitis may be idiopathic, parasitic or part of CSS - reported following treatment with Montelukast but never with eosinophilic mastitis. Cardiac masses in CSS have been reported occasionally in adults, but not previously seen in children.
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Open Access This article is published under license to BioMed Central Ltd. This is an Open Access article is distributed under the terms of the Creative Commons Attribution License ( https://creativecommons.org/licenses/by/2.0 ), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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Cite this article
Baildam, E., Pain, C., McErlane, F. et al. Unusual presentations of Churg Strauss syndrome in paediatrics: a tale of 2 masses from one centre experience. Pediatr Rheumatol 9 (Suppl 1), P96 (2011). https://doi.org/10.1186/1546-0096-9-S1-P96
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DOI: https://doi.org/10.1186/1546-0096-9-S1-P96
Keywords
- Rhinitis
- Mastitis
- Maxillary Sinus
- Pericardial Effusion
- Montelukast