ISSN: 2161-1149 (Printed)
Luton and Dunstable University Hospital, UK
Posters & Accepted Abstracts: Rheumatology
A 46 year old gentleman of Bangladeshi origin presented to the emergency department with a three week history of bilateral loin pain, worse in the left loin. Clinical examination elicited left renal angle tenderness. The abdomen was otherwise soft with no signs of peritonism. The clinical impression initially was that of renal calculi and pyelonephritis; however it was noted that there were atypical features against either diagnosis. He proceeded to have a CT abdomen with contrast which showed a soft tissue density thickening along the left postero-lateral margin of the descending aorta. This was radiologically suspected to represent acute left infra-renal periaortitis which would account for the patient's symptoms. A subsequent CT PET confirmed increased activity in the aortic soft tissue. The patient had significantly raised ESR and CRP. In addition, IGG4 serology was performed and was strongly positive. Upon review by the rheumatology team, the patient was commenced on oral steroids and low dose azathioprine with dramatic improvement in his symptoms within 2-3 days. He was diagnosed with IGG4-related periaortitis. The patient has since been followed up in rheumatology outpatients with gradual tapering of his immunosuppressants and regular review of symptoms and clinical condition. IGG4-related disease has recently been documented and acknowledged as a clinical entity since early 2000. Since then further cases of IGG4 disease have been described, however the diagnosis remains rare. IGG4-related disease is an immunemediated condition characterized by a lymphoplasmacytic infiltrates enriched in IgG4 plasma cells, affecting various different organs. Clinical features can vary considerably, depending on the organ involved. Commonly IGG4-related disease can cause autoimmune pancreatitis, retroperitoneal fibrosis, aortitis or periaortitis, thyroid disease, lung and pleural disease, and IGG4 related sclerosing cholangitis. Periaortitis classically presents with dull abdominal and flank pain of insidious onset. Systemic features such as fever, weight loss, malaise and vomiting may also be present. There is limited data on the natural history of the disease. Typically, patients display a fast recovery with immunosuppressants. However, relapses are common with discontinuation of therapy. Current recommendations for treatment are prednisolone 40 mg/day as first line. Second line therapies, or in those where glucocorticoidsparing treatment is required, azathioprine and mycophenolate mofetil are reasonable choices. Rituximab is also a viable second line option if available. The prognosis in IGG4-related disease is highly variable. Significant organ damage however, can result from uncontrolled and progressive inflammatory disease.
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