title: Mixed connective tissue disorder and Castleman's disease.
abstract: We present a 16-year-old girl who presented with polyarthritis in association with Raynaud's phenomenon, malar rash, oral ulcers, photosensitivity and alopecia of 6 months duration. On evaluation, it emerged that she had a mixed connective tissue disorder with a mesangio-proliferative glomerulonephritis. Her Chest radiograph revealed a well defined left mid and lower zone opacity with evidence of a hilar mass on CT Thorax. Histopathological examination following CT guided biopsy of the mass revealed a hyaline vascular type of Castleman's disease. Mixed Connective Tissue Disorder with Castleman's Disease is a rare association; the patient presenting with varied and interesting manifestations. It is important to understand this association in view of management. The exact etio-pathogenesis of the autoimmune manifestations in patients with Castleman's disease is not clear. Treatment with immunosuppression can suppress both immune manifestations and result in tumour regression as well.
authors.affiliation: Department of Medicine, Christian Medical College, Vellore.
authors.forename: Anugrah
authors.initials: A
authors.lastname: Chrispal
authors.forename: Zoya
authors.initials: Z
authors.lastname: Vasuki
authors.forename: Elsa Mary
authors.initials: EM
authors.lastname: Thomas
authors.forename: Hari Kishan
authors.initials: HK
authors.lastname: Boorugu
mesh_terms: D000293:Adolescent; D001379:Azathioprine / Q000627:therapeutic use; D001706:Biopsy; D005871:Castleman Disease / Q000150:complications / Q000473:pathology*; D005260:Female; D015432:Glomerulonephritis, Membranoproliferative / Q000473:pathology*; D006801:Humans; D006886:Hydroxychloroquine / Q000627:therapeutic use; D008947:Mixed Connective Tissue Disease / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D011239:Prednisolone / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D006886:Hydroxychloroquine; D011239:Prednisolone; D001379:Azathioprine