Idiopathic fibrosclerosis presenting as angioedema and upper airway obstruction

Abstract

Rationale Retroperitoneal fibrosis is uncommon condition of mostly unknown etiology. The clinical presentations are variable from constitutional symptoms to rare association of other fibroinflammatory diseases such as sclerosing cholangitis, mediastinal fibrosis and parotid gland. With such a broad array of presenting symptoms, the early stage of the disease can mimic several other conditions. In this report, we describe an idiopathic fibrosclerotic patient who came into the hospital for prolonged fever, fatigue, weight loss and abdominal pain then later found to have facial and neck edema with trimus. Upon the investigations of abdominal CT-scan and retrograde pyelography revealing bilateral hydronephrosis with UPJ obstruction and hypodensity lesion between posterior bladder and anterior rectum together with nasopharynx MRI demonstrating infiltrative lesions involving right parapharyngeal, parotid and were submandibular spaces, subsequently, the diagnosis of retroperitoneal and mediastinal fibrosis made. Nasopharynx, oropharynx and submandibular biopsy also showed chronic inflammation with fibrosis and loose connective fibrous tissue, respectively. Other investigations revealed ANA of 1:40 titer, alkaline phosphatase of 393 u/L, hypoalbuminemia of 2.4 mg/dL. The rest of laboratory examination including ANCA, cryoglobulin test and anti HIV were negative. Endotracheal tube was placed to prevent obstruction. High dose of oral corticosteroid and tamoxifen were started. Systemic steroid was tapered down to 0.5 mg/kg/day in 8 weeks. At a follow up of 4 month, she continues to do well. Conclusion In summary, Idiopathic fibrosclerosis can present as angioedema and impending airway obstruction accompanying with constitutional and urinary symptoms will lead us to recognize retroperitoneal fibrosis as well.