Journal of the Royal Society of Medicine Volume 76 December 1983 1023 Variable symptomatology in idiopathic retroperitoneal fibrosis

C D M Fletcher MB BS2 P E M Jarrett MA FRCS Kingston Hospital, Kingston upon Thames, Surrey KT2 7QB

Summary: Idiopathic retroperitoneal fibrosis is uncommon enough to be placed low down on a list of differential diagnoses of any problem facing a general surgeon in a district hospital. In this paper, 4 cases of retroperitoneal fibrosis are described, all of whom presented within a 5-year period to the same surgeon; each described a different symptomatology. This paper serves to reiterate the diversity of presenting features in retroperitoneal fibrosis and to demonstrate that this condition may not be as rare as is widely believed. Introduction Idiopathic retroperitoneal fibrosis is an unusual condition, first described in the English literature by Ormond in 1948. It classically affects middle-aged men, who most often present with loin or back pain (Osborn et al. 1981); it results in ureteric obstruction, with or without affecting the neighbouring aorta and inferior vena cava. Most cases can be effectively treated by ureterolysis, although steroids may be indicated on occasion (Abercrombie & Vinnicombe 1980). Published series of retroperitoneal fibrosis have very rarely exceeded 15 cases, even from large university hospitals over ten-year periods (Saxton et al. 1969, Lepor & Walsh 1979, Larrien et al. 1980). This report describes 4 cases from a district general hospital, all presenting within a 5-year period. Case reports Case 1: A 44-year-old man presented in December 1977 complaining of blurred vision, vomiting and weight loss. He was found to have a blood pressure of 200/120 mmHg and bilateral papilloedema. Malignant hypertension was diagnosed and treated with hydrallazine and diazoxide. Biochemical tests revealed a serum urea of 20.6 mmol/l (124 mg/100 ml) and potassium of 4.0 mmol/l, with heavy proteinuria. Investigation of his chronic renal failure revealed bilateral external compression of the at the level of the fifth lumbar vertebra. Laparotomy revealed retroperitoneal fibrosis and bilateral ureterolysis was performred. Renal function progressively improved over the subsequent 4 years. He remains well with normal blood biochemistry and requires no antihypertensive medication. Case 2: A 56-year-old man presented in September 1978 complaining of nonspecific lower abdominal pain. An intravenous (IVP) revealed an ill-defined filling defect at the base of the bladder, the source of which was unclear, with no other abnormality. was negative. Renal function tests were normal. He returned 4 months later with malaise, vomiting and weight loss and was found to have a serum urea of 26.6 mmol/l (160 mg/100 ml), creatinine 540 Mmol/l (7.0 mg/100 ml) and potassium 6.3 mmol/l. His hyperkalaemia was corrected with calcium resonium enemas: repeat cystoscopy with retrograde ureterograms revealed bilateral hydronephrosis with bilateral ureteric obstruction

'Accepted 28 July 1983 2Present address: Department of Histopathology, St Thomas's Hospital, London SEl 7EH 0141-0768/83/121023-03/$01.00 kl.&)1983 The Royal Society of Medicine 1024 Journal of the Royal Society of Medicine Volume 76 December 1983 at the level of the fourth lumbar vertebra. Laparotomy showed retroperitoneal fibrosis extending from the second to the fifth lumbar vertebrae. Bilateral ureterolysis was performed and by September 1980 there was minimal radiological evidence of hydronephrosis. At present he is well and has normal serum biochemistry. Case 3: A 59-year-old man presented in May 1980 complaining of nausea, vomiting, weight loss and a repeatedly painful red right leg. He was found to have severe thrombophlebitis. Serum biochemistry revealed a urea of 23.7 mmol/l (142.7 mg/100 ml), creatinine of 545 imol/l (7.1 mg/100 ml) and potassium of 4.6 mmol/l. Haemoglobin was 8.9 g/dl. IVP showed bilateral hydronephrosis. Investigation of his chronic renal failure was repeatedly complicated by four, separate, deep vein thromboses in the calves and one pulmonary embolus. Eventually, descending nephroureterograms were performed through tubes; bilateral external ureteric compression, extending 2 cm, at the level of the upper sacrum was demonstrated. Laparotomy confirmed the clinical diagnosis of retroperitoneal fibrosis, extending from the sacrum to the third lumbar vertebra. Bilateral ureterolysis was performed, and since that time the patient has remained well with only marginally elevated serum urea and creatinine. Case 4: A 58-year-old man presented in February 1982 complaining of acute pain and swelling of the left leg. In 1979 he had been extensively investigated for backache and abdominal pain but no diagnosis had been made. For 5 years he had suffered intermittent claudication, with weak femoral and absent popliteal and dorsalis pedis pulses, which had not responded to medical treatment. Venography revealed very extensive thrombosis of the left iliofemoral vein. He was anticoagulated. Numerous investigations including full blood count, urea and electrolytes, sigmoidoscopy, barium enema, barium meal and abdominal ultrasound revealed no significant abnormality. However, IVP disclosed a right hydronephrosis -with hydroureter and retrograde ureterograms showed smooth external compression of the middle third of the right . Laparotomy confirmed the diagnosis of retroperitoneal fibrosis and bilateral ureterolysis was performed. He has no further claudication, normal pulses in both legs, no backache or abdominal pains and remains completely well. Periuretic tissue from each of the four cases was biopsied and examined histologically: all showed the classical appearance of idiopathic retroperitoneal fibrosis, manifest as a nonspecific chronic inflammatory and fibrotic process. Discussion These four cases demonstrate a wide spectrum of presenting features in retroperitoneal fibrosis, and serve to remind the general surgeon that this condition may not be as rare as is often thought. The diversity of symptoms, most of which are not directly referable to the urinary tract, makes this condition a diagnostic problem to the non-specialist. Retroperitoneal fibrosis should always be considered since it is surely the most treatable cause of chronic renal failure, and the results of treatment in our and other series (Lepor & Walsh 1979) are generally rewarding. Proteinuria in retroperitoneal fibrosis (as seen in Case 1), in the absence of urinary tract infection, is unusual: in fact uraemia with a normal urinalysis is said to be a useful diagnostic aid in this condition (Lemmon & Kiser 1966). However, it is clear that if malignant hypertension supervenes then both glomerular and renal arteriolar damage will occur, such that proteinuria is inevitable. None of these patients had a predisposing drug history of known relevance, nor did they suffer any associated systemic manifestations such as a connective tissue disease. All had an elevated erythrocyte sedimentation rate and raised fibrinogen levels (compared with age- and sex-matched controls), but other tests of fibrinolysis were normal. All four patients had positive smooth muscle autoantibodies, ranging in titre from 1 in 40 to 1 in 80. This finding Journal of the Royal Society of Medicine Volume 76 December 1983 1025 is of unknown significance and has been reported once before (Saxton et al. 1969): while this may support an autoallergic aetiology (British Medical Journal 1981), it is unclear whether idiopathic retroperitoneal fibrosis is a reaction to a damaged aortic wall (Mitchinson 1970) or to other smooth muscle, perhaps of the ureters. This would seem worthy of further study. Acknowledgment: The authors would like to thank Dr J R O'Brien, Honorary Consultant Haematologist at Saint Mary's Hospital, Portsmouth, for performing the fibrinolytic studies. References Abercrombie G F & Vinnicombe J (1980) British Journal of Urology 52, 443-445 British Medical Journal (1981) 282, 1343 Larmen A J, Weiner I, Abston S & Warren M (1980) Surgery, Gynecology and Obstetrics 150, 699-702 Lemmon W T & Kiser W S (1966) Journal of Urology 96, 658-667 Lepor H & Walsh P C (1979) Journal of Urology 122, 1-6 Mitchinson M J (1970) Journal of Clinical Pathology 23, 681-689 Ormond J K (1948) Journal of Urology 59, 1072-1079 Osborn D E, Rao P N, Barnard R J, Ackriil P, Ralson A J & Best J J K (1981) British Journal of Urology 53, 292-296 Saxton H M, Kilpatrick F R, Kinder C H, Lessof M H, McHardy-Young S & Wardle D F H (1969) Quarterly Journal of Medicine 38, 159-181