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Retroperitoneal fibrosis is a rare condition characterized by chronic inflammation and marked fibrosis of the retroperitoneal tissue, often leading to entrapment of abdominal organs. We report a 69-year-old white man who presented with a 5-week history of gradual onset of progressive abdominal distension. He had no history or risk factors for an underlying liver condition. Ascites and a retroperitoneal mass encasing the major abdominal vessels were revealed on imaging. Biopsies of the mass confirmed the presence of retroperitoneal fibrosis, and the ascitic fluid was milky, consistent with chylous ascites. We discuss this rare presentation and the challenges of treatment for chylous ascites caused by RPF, including the role for supportive treatment.

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Background/Aims  Henoch-Schonlein purpura (HSP) is a small vessel vasculitis characterised by IgA deposition. Adrenal haemorrhage has been reported previously in HSP, but is rare. Methods  A 33-year-old lady was admitted with myalgia, pyrexia, a faint petechial rash on her legs, a CRP of 92mg/L (<5). No signs of meningism were found. A throat swab for viral pathogens (taken prior to the Covid-19 pandemic) returned negative, and blood culture showed no growth. Within a few days she returned to the Accident & Emergency Department with worsening of the rash but was again discharged. The following week she was admitted with abdominal pain and a prominent purpuric rash on her legs. Results  Full blood count, Rheumatoid Factor, Anti-Nuclear Antibodies, ANCA and Anti-Glomerular Basement Membrane Antibodies, serum Tryptase & C1 esterase inhibitor were all within normal limits. Urine culture grew E.coli. Complement C3 was raised at 2.24 g/L (0.75-1.65) and C4 was within normal range. Her blood pressure was raised at 173/104 mmHg. Antistreptolysin O serology was also normal. Urine analysis revealed haematuria and mild proteinuria. Urine Protein:Creatinine ratio of 22mg/mmol (normal <50). Her purpuric rash had progressed, and she had further abdominal pain. She had taken co-codamol which led to nausea & constipation. She had avoided NSAIDs. Her CRP by this stage was 131mg/L. Clinically the rash on the legs, abdominal pain and haematuria was consistent with HSP. She was started on Prednisolone 10mg od, tapering in 2.5mg per week decrements. The patient was discharged home but returned the following weekend with cramping abdominal pain radiating to the back. Abdominal Xray showed dilated colon at the splenic flexure. After review by the Surgical on call team, a CT Abdomen & Pelvis showed a right adrenal haemorrhage, and inflammatory change in both adrenals. Although she did not display clinical signs of Addisonian crisis or sepsis, she was transferred to HDU for monitoring. Her blood pressure was 151/86 mmHg and her serum urea & electrolytes were normal. The Prednisolone dose was changed to Hydrocortisone, and the dose increased to cover stress of acute illness. MRI of the adrenal glands confirmed right adrenal haemorrhage but showed no evidence of aneurysms or tumours. Antiphospholipid & Cryoglobulins screens were negative. Further blood cultures, pneumococcal and legionella antigens returned negative. Viral Hepatitis screen (A, B & E) was negative. Synacthen test revealed a suboptimal response (Cortisol t0=181nmol/L, t30=260nmol/L, normal response >420nmol/L). Adrenal Autoantibodies returned negative. She was discharged home on oral hydrocortisone and fludrocortisone and remained well at Rheumatology and Endocrine follow up. Conclusion  Adrenal haemorrhage is a rare complication of HSP and should be considered as part of the differential diagnosis in such patients presenting with acute abdominal pain and vasculitic rash. Disclosure  J.G.C. Shek: None. M. Curuvija: None. S. Al-Mudhaffer: None. G. Das: None. E.P.M. Humphreys: Honoraria; E.H. has participated in advisory board meetings for Pfizer. Other; E.H. has received sponsorship to attend conferences from Abbvie, UCB and Celgene.

Background18F-Fluorodeoxyglucose positron emission computerised tomography (FDG-PET-CT) is commonly used to aid the diagnosis of large vessel vasculitis (LVV). Its precise role and sensitivity during follow-up of LVV remains to be defined, and use is typically restricted to investigation of an unexplained acute phase response. We have encountered an additional limitation in LVV patients with synthetic arterial grafts, namely interpretation of FDG uptake confined to the graft. This is typically reported as vasculitic activity or infection, even in the absence of supportive clinical data.ObjectivesTo investigate non-specific uptake of FDG by synthetic arterial grafts in LVV.MethodsTwelve patients with FDG-uptake localised to arterial grafts were identified, 10 with LVV, and 2 with non-inflammatory aortopathy. The maximum standardised uptake values (SUV) for the grafts were quantified. The intensity of uptake by each graft and the native aorta was scored against constitutive hepatic uptake. Time after surgery, C-reactive protein, and infection screen results were obtained. Disease progression was estimated by magnetic resonance angiography (MRA).ResultsTwelve patients (10 female, mean age 41.4 yrs, range 23-60) who had undergone graft surgery and FDG-PET scanning were identified, 8 with Takayasu arteritis (TA), 2 giant cell aortitis, 1 Marfan's syndrome and 1 non-inflammatory aortic wall degeneration (Table). The index case (patient 1) presented with fatigue 2 years post-surgery. An FDG-PET-CT demonstrated increased FDG uptake specifically localised to the graft site, raising concerns regarding active LVV or graft infection. However, blood indices including leukocyte count and acute phase reactants were normal, and repeat blood cultures revealed no infection. Study of nine other LVV patients revealed directly comparable findings (Table). The 2 patients with non-inflammatory disease also demonstrated significant uptake in aortic grafts, with no identifiable cause. The mean time post-surgery to first scan was 40 months (range 6-90). The mean maximum SUV of the grafts was (4.29±1.22). The uptake score in the grafts and native aorta relative to the liver were 2.83±0.39 versus 0.42±0.67 respectively (p<0.001). The CRP was not raised (mean ± SD 5.95±4.82, NR<10 mg/L), no infections were identified. Eight patients had repeat FDG-PET-CT scans with similar graft specific uptake identified. In 9 patients treatment was left unchanged. In 2 cases immunosuppression was increased and one received antibiotics, both without any change in FDG uptake. In those with LVV, immunosuppression has been subsequently weaned/withdrawn in all cases with no disease progression at 76.5 months (range 9-180).ConclusionsAlthough FDG-PET-CT scanning has proved useful for the diagnosis of active LVV, its role in the follow-up of patients on treatment remains undecided. FDG uptake confined to synthetic graft sites in LVV does not necessarily equate to infection or active vasculitis requiring treatment escalation. Uptake may represent a macrophage-drive...