HIV infection presenting as renal polyarteritis nodosa

We present a case of renal vasculitis (polyarteritis nodosa) in a 36-year-old female prostitute having undetected HIV infection (AIDS-related complex) and chronic B-hepatitis. The pathogenesis and significance of the association between these entities are reviewed.     

Ureteric bupivicaine infusion for loin pain haematuria syndrome

INTRODUCTION

Loin pain haematuria syndrome is a common problem with complications including opiate dependence. Morbidity treatments include intra-ureteric capsaicin infusion, nephrectomy, autotransplantation and nephrolysis. We explored the use of flexible cystoscopic infusion of intra-ureteric bupivicaine.

PATIENTS AND METHODS

Patients presenting with chronic loin pain underwent urological and nephrological evaluation. Bupivicaine (0.5%, 20 ml) was infused via an intra-ureteric catheter under flexible cystoscopic guidance. Repeat infusions were offered if indicated.

RESULTS

Sixteen of 17 patients with 1-year follow-up responded and were satisfied. Twelve of these required repeat infusions (mean, 2.9 infusions). The procedures were well tolerated by all patients without adverse effects.

CONCLUSIONS

Intra-ureteric bupivicaine infusion has a place in the management of patients with chronic renal pain. It offers a minimally invasive alternative to other treatments. This procedure warrants further investigation within a randomised, controlled trial setting.     

Intra-ureteric capsaicin in loin pain haematuria syndrome: efficacy and complications

OBJECTIVE: To evaluate the safety and efficacy of intra-ureteric capsaicin for loin pain haematuria syndrome (LPHS). PATIENTS AND METHODS: In an open prospective pilot study, four middle-aged patients (three women and one man) with LPHS resistant to therapies such as splanchnic nerve block, psychological treatment or renal autotransplantation (one) were assessed. An intra-ureteric infusion of capsaicin (30 mg/100 mL of 30% alcohol in saline) for 30 min with bladder irrigation was administered under general anaesthesia, with a subsequent intravenous patient-controlled narcotic analgesic pump for pain control. Double-concentration capsaicin was used for second infusions, if necessary when the response to the earlier infusion was inadequate or incomplete. RESULTS: The first patient had experienced reduced pain levels for the first 3 months only, with no benefit from the subsequent treatments with higher doses of capsaicin (60 mg). The second patient with recurrent pain in an autotransplanted kidney had no benefit from either a 30 or 60 mg capsaicin infusion a month apart, but developed a fibrotic stricture at the transplant pelvi-ureteric junction, requiring pyelocystoplasty. The third patient with concurrent depression had no benefit from a 30-mg infusion of capsaicin. The fourth patient experienced no pain relief from a 30 mg infusion of capsaicin but developed proteinuria secondary to mesangial proliferative glomerulonephritis, ureteric inflammation needing stenting within 7 days of treatment and subsequently nephrectomy for a nonfunctioning kidney at 3 months. CONCLUSION: Intra-ureteric capsaicin was neither effective nor safe in LPHS; the contribution of the alcohol diluent cannot be excluded.     

Polyarteritis nodosa and cutaneous polyarteritis nodosa

Cutaneous vasculitis can present with a variety of lesions and associated symptoms. It is important for the clinician to be able to recognize vasculitis lesions and know when to pursue further laboratory studies that will determine the extent of the disease process in a patient. This review compares and contrasts polyarteritis nodosa with its cutaneous counterpart. The diagnostic workup and management of these two entities as well as the overall approach to a patient with a possible vasculitic condition are emphasized.     

The outcome of renal denervation for managing loin pain haematuria syndrome

OBJECTIVE: To evaluate the outcome of renal denervation for the treatment of loin pain-haematuria syndrome (LPHS), a rare syndrome of unknown cause associated with debilitating and intractable loin pain. PATIENTS AND METHODS: The case notes of 32 patients having 41 renal denervations were reviewed. Data collected included patient demographics, possible causes, cure or not after renal denervation, time to recurrence of pain after denervation and further operative intervention for managing LPHS. RESULTS: Full data were available for 24 patients (13 women; median age 43 years, range 28-74) having 33 denervations (eight bilateral and one repeat) with a median follow-up of 39.5 months. Most patients had no identifiable underlying cause although many had initially been diagnosed as having stone disease (42%) or pyelonephritis (25%), but with no corroborative evidence. All patients had been extensively investigated and had normal urine samples and cytology, and no abnormality on intravenous urography, renal tract ultrasonography and isotopic renography. Twenty-four renal denervations (73%) were followed by recurrent ipsilateral pain at a median (range) of 11 (0-120) months after surgery. Nine denervations (25%) in six men and two women were curative (median follow-up 16.5 months). Of those with recurrent pain, nine (38%) proceeded to nephrectomy, of whom three then developed loin pain on the contralateral side and two developed disabling wound pain. The analgesic requirement was less after eight non-curative denervations. There were no significant postoperative complications. CONCLUSIONS: Renal denervation has only a 25% success rate for managing pain associated with LPHS and should be used cautiously for this indication. Men had more benefit from the treatment; a third of patients had less requirement for analgesic after non-curative denervation.     

The natural history and management of the loin pain/haematuria syndrome

This is a rare, but potentially very troublesome disorder. We have been able to carry out detailed radiological, histological and biochemical investigations on 51 patients and the results of these investigations are presented. Considerable evidence of abnormal platelet activity has been found. The histological evidence has come from both biopsy and nephrectomy specimens and has shown aggressive atherosis leading to microembolic lesions and in some cases showing microaneurysm formation. In some instances this has led to areas of infarction both small and large. Most of the cases studied have been fully followed up and it has been found that despite the histological changes, the blood pressure remains normal and no demonstrable deterioration in renal function occurs. Attempts at treatment are described and discussed.     

Early experience of intra-ureteric capsaicin infusion in loin pain haematuria syndrome

OBJECTIVES: To evaluate early results of the intra-ureteric instillation of capsaicin for the treatment of loin pain haematuria syndrome (LPHS). PATIENTS AND METHODS: Ten patients with LPHS were treated using intra-ureteric capsaicin instillation. A solution of capsaicin was infused into the affected ureter through an embolectomy catheter, under anaesthesia. The success of the treatment was assessed using patient questionnaires and the quantitative reduction in the patients' analgesic requirements measured. RESULTS: During a mean follow-up of 6 months, six of the 10 patients had short- to medium-term symptomatic relief after one or more treatments; four had no relief from their symptoms. One patient had a mucosal ulceration in the bladder after extravasation of the capsaicin solution. Two patients subsequently underwent simple nephrectomy for symptomatic nonfunctioning kidneys. CONCLUSION: These results are consistent with other preliminary reports of the efficacy of capsaicin treatment in LPHS and such treatment therefore has a definite therapeutic role in this difficult condition. We are uncertain if the treatment contributed to the deterioration of the excised kidneys. This early experience suggests a need for careful consideration when contemplating this treatment, with attention directed to both the initial diagnosis and possibly the technique of capsaicin/instillation. We include a protocol to follow when preparing patients for capsaicin treatment.     

Testicular pain: an unusual presentation of polyarteritis nodosa

We report a case in which the initial presentation of polyarteritis nodosa was alternating bilateral orchialgia. The diagnosis was confirmed by testicular biopsy. Testicular biopsy is an uncommonly reported diagnostic procedure despite the high incidence of testicular involvement in polyarteritis nodosa. We emphasize the value of testis biopsy, particularly when biopsy of other sites does not prove the diagnosis in suspect cases.     

A case of polyarteritis nodosa presenting as a mass in scrotum

A 16-year-old boy with a painful tumor in the left scrotum was referred to our department. CT scans showed a low density area in the left testis, so we diagnosed a left testicular tumor and performed left inguinal orchiectomy. Histological examination revealed polyarteritis nodosa (PN) of the testis and epididymis. Systemic examination revealed no other evidence of PN. Although induration developed in the right epididymis after the operation, it resolved with steroid therapy. The patient is currently asymptomatic and is being followed at our clinic. The pathogenesis and management of this rare condition are discussed.     

Renal failure associated with polyarteritis nodosa

We reported 5 patients with renal failure associated with polyarteritis nodosa (PN). In all patients the renal dysfunction became apparent from 2 to 4 weeks after the onset of fever or neuromuscular symptoms with laboratory findings supporting marked inflammatory process and took the course of either acute or rapidly progressive renal failure. The clinical diagnoses on admission were variable; fever of unknown origin, peptic ulcer disease, polyneuritis multiplex, disseminated intravascular coagulation and Buerger's disease. Microscopic examination of the kidneys in 3 by renal biopsy and in 2 by autopsy revealed crescentic glomerulonephritis without immunoglobulin deposit, segmental necrotizing glomerulonephritis, marked inflammatory infiltrate around the glomeruli, granulomatous lesion, tubular necrosis and cortical necrosis other than necrotizing vasculitis. The outcomes were favorable with prednisolone monotherapy in two patients who had biopsy-proven diagnosis and treatment early in the course. Of all PN the prevalence of renal involvement is considered to be about 70%, the prognosis of which has been considered very poor. However it seems possible to alter fatal outcome of this disease by starting treatment with confident histological proof early in the disease process.     

Isolated testicular polyarteritis nodosa

Isolated polyarteritis nodosa is a rare condition that may be triggered by local exposure to certain agents capable of provoking local immunostimulation without a systemic immune reaction. Isolated polyarteritis nodosa in the testes presents similar histologic characteristics as those of systemic inflammation, although infarcted areas in the testis are more common in generalized polyarteritis nodosa. Definitive diagnosis requires histologic study. We present the case of a 26-year-old patient with isolated testicular polyarteritis nodosa whose symptoms consisted predominantly of intense testicular pain and slight enlargement of both testes.     

Renal angiography in children with polyarteritis nodosa

This study describes the angiographic findings in children with polyarteritis nodosa (PAN). Visceral angiograms of 25 children with PAN were reviewed retrospectively by two independent radiologists. In the PAN group, 40% of children had aneurysms demonstrated on selective renal angiography. Most aneurysms affected small and medium-sized arteries. There was agreement between radiologists regarding medium and large aneurysms (K=0.81), but less so for smaller aneurysms. Overall, the presence of medium or large aneurysms was significantly associated with the presence of renal impairment and hypertension. Non-aneurysmal changes were detected more commonly on renal angiography than aneurysms in the PAN group. The most reliable non-aneurysmal signs were perfusion defects, the presence of collateral arteries, lack of crossing of peripheral renal arteries, and delayed emptying of small renal arteries. The sensitivity and specificity of renal angiographic diagnosis of PAN using aneurysms alone was 43% (SE 10%) and 69% (SE 14%) respectively. The sensitivity increased to 80%, and specificity fell to 50% for angiogram positivity defined as the presence of at least one of the most reliable non-aneurysmal signs irrespective of the presence of aneurysms. Aneurysms were also demonstrated on hepatic and mesenteric angiography, and non-aneurysmal signs were found on hepatic, mesenteric, and splenic angiography, although interobserver agreement for angiographic findings in these vascular beds was lower. It is important to consider both aneurysmal and non-aneurysmal angiographic signs, and to include examinations of several vascular beds when utilising angiography for diagnostic purposes in children with PAN. Received: 6 August 2001 / Revised: 19 December 2001 / Accepted: 19 December 2001     

Thrombotic thrombocytopenic purpura associated with polyarteritis nodosa

We present a case of classical polyarteritis nodosa (PN) overlapping thrombotic thrombocytopenic purpura (TTP). A 70-year-old woman was transferred to our hospital because of general fatigue and fever. On admission, laboratory findings revealed leukocytosis, normochromic normocytic anemia and renal dysfunction. About one week later, she developed disturbance of consciousness, and laboratory findings revealed rapidly progressive thrombocytopenia and renal dysfunction. We suspected the presence of microscopic polyangiitis (MPA), based on mild elevation of myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibody (ANCA). On post-admission Day 11, renal biopsy was performed but the diagnosis of MPA could not be confirmed because of the absence of glomerular crescent formation or vasculitis. However, the biopsy specimen showed many collapsed glomeruli and interstitial inflammation, indicating the presence of occlusive lesions, such as vasculitis in larger arteries. We instituted methylprednisolone pulse therapy, cyclophosphamide and plasma exchange, because the clinical symptoms also satisfied the criteria of TTP. Despite the intensive treatment, the patient died on 43rd day of hospitalization due to thalamic hemorrhage. Autopsy showed typical findings of classical PN including disruption of arterial walls and fibrinoid necrosis in the medium-sized arteries of the kidneys and colon. We detected reduced activity of von Willebrand factor-cleaving protease (VWF-CP) and the presence of plasma inhibitory IgG against VWF-CP. A better understanding of the mechanisms would be useful.