P147 Venous fibrosis and stenosis in IgG4-related disease

Abstract

Abstract Background/Aims IgG4-related disease (IgG4-RD) is a multi-system fibro-inflammatory disease. Common presentations involve salivary and lacrimal glands, orbital disease, autoimmune pancreatitis, retroperitoneal fibrosis and tubulointerstitial nephritis. The main histopathological features are a dense, polyclonal, lymphoplasmacytic infiltrate rich in IgG4+ plasma cells, storiform fibrosis and obliterative phlebitis. Cases of retroperitoneal fibrosis resulting in venous compression with DVT are well described, however to our knowledge venous fibrosis and stenosis confirmed with IVUS in IgG4-RD RPF has never been described. Methods An 82-year-old lady presented with bilateral lower leg oedema. She had a past diagnosis of IgG4 disease made on the basis of retroperitoneal fibrosis (confirmed on PET-CT), raised serum IgG4 levels (IgG4 2.38; normal 0.04-0.86 g/l) a supportive tissue biopsy (fibro-lymphocytic tissue with non-diagnostic IgG4 plasma cells) and raised inflammatory markers (CRP 104, ESR 120). Disease responded well to prednisolone and azathioprine, resulting in regression of RPF. She presented with left leg pitting oedema 4 months after presentation which progressed to chronic leg venous ulceration. D-dimer testing, albumin, liver function, and renal function tests were normal. There were no signs of lymphoedema. Venous Doppler studies of the left lower leg showed a patent and compressible left common femoral, superficial femoral and popliteal vein excluding a DVT and Baker’s cyst. A repeat CT pelvis showed no compressive mass. An intravascular ultrasound showed that the wall of the IVC was stiff and of dampened amplitude with respiratory motion. There was also increased echogenicity of the left common and external iliac vein wall with significant stenosis of the distal left common iliac vein and the external iliac veins. On the right side, there was increased echogenicity of the right common iliac vein, but the lumen was well preserved. Results These findings indicated fibrosis of the IVC wall and left common iliac vein with resulting venous stenosis. The patient underwent bilateral iliac vein stenting with improvement of leg swelling and eventual healing of the venous ulcer. Conclusion This case demonstrates that venous fibrosis and stenosis may occur in the absence of compressive lesions leading to leg swelling. Further diagnostic information may be identified by IVUS and radiological intervention with venous stents may be required. Disclosure R. Baslas: None. D. Condurache: None. D. Greenstein: None. L. Patrone: None. S. Hamdulay: None.