Longterm outcome of Wegener's granulomatosis in patients with renal disease requiring dialysis.
ABSTRACT It is known that renal failure is a poor prognostic marker for survival in Wegener's granulomatosis (WG). We investigated the longterm outcome of patients with WG who have severe renal disease requiring dialysis.
We performed a retrospective analysis of 104 patients with WG followed at our institution between 1982 and 1997. Twenty-three patients who required dialysis were studied in detail to determine outcomes and factors that influenced survival and restoration of renal function.
Of 23 dialysis dependent patients with WG, 11 died (Group 1). 7 either remained dialysis dependent or received successful renal transplants (Group 2), and 5 substantially recovered renal function (Group 3). Mean serum creatinine at the end of a mean followup period of 38.4 months for Group 3 was 1.8 mg/dl. There was no apparent difference between groups in regard to disease profile, e.g., distribution of organ involvement or serum creatinine when renal impairment was first recognized (mean serum creatinine for groups: 1: 3.0 mg/dl; 2: 5.6 mg/dl; 3: 5.5 mg/dl) and peak serum creatinine prior to dialysis (means for groups: 1: 9.5 mg/dl; 2: 10.5 mg/dl; 3: 9.6 mg/dl). Infection secondary to immunosuppression was the leading cause of death in Group I patients.
Because the clinical profile and degree of renal failure, as judged by serum creatinine, did not differ among patients who did or did not regain dialysis independent renal function, we recommend aggressive immunosuppressive therapy in all cases of active WG with acute rapidly worsening renal failure, regardless of the severity of renal impairment.
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ABSTRACT: To estimate the incidence of and survival rates for WG, microscopic polyangiitis (MPA), Churg-Strauss syndrome (CSS) and PAN within a defined population in southern Sweden. Cases were retrieved using hospital records and a serology database. All new cases of WG, MPA, CSS and PAN between 1997 and 2006 were included, provided they met pre-defined criteria, and were followed until 30 June 2008. The study area comprised two health care districts with a total population of 641 000. The standardized mortality ratio (SMR) was estimated using Swedish population data as a reference. A total of 140 (WG, 63; MPA 65; CSS 6; and PAN 6) cases (52% women) with a median age of 67.6 (range 20-96) years fulfilled the inclusion criteria. The annual incidence per million of the population (95% CI) was estimated to be 9.8 (7.4-12.2) for WG, 10.1 (7.7-12.6) for MPA and 0.9 (0-1.7) for both CSS and PAN. The highest incidence was found in patients aged >or=75 years (79.1/million). The 1- and 5-year survival rates were 87.8 and 71.6% for all patients, but lower for MPA (80 and 55%) compared with WG (95 and 83%; P = 0.001), although the difference was not significant in the multivariate analysis. The SMR was 2.77 (95% CI 2.02, 3.71) for all patients. The incidence of WG and MPA was equal in our district, but there was a difference in survival rates related to age and renal function. A progressive increase in age-specific incidence rates was observed.Rheumatology (Oxford, England) 10/2009; 48(12):1560-5. · 4.24 Impact Factor
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ABSTRACT: Numerous anatomical and functional changes occurring in the aging kidney lead to reduced glomerular filtration rate, lower renal blood flow and impaired renal autoregulation. The elderly are especially vulnerable to the development of renal dysfunction and in this population acute renal failure (ARF) is a common problem. ARF is often iatrogenic and multifactorial; common iatrogenic combinations include pre-existing renal dysfunction and exposure to nephrotoxins such as radiocontrast agents or aminoglycosides, use of NSAIDs in patients with congestive cardiac failure and use of ACE inhibitors and diuretics in patients with underlying atherosclerotic renal artery stenosis. The aetiology of ARF is classically grouped into three categories: prerenal, intrinsic and postrenal. Prerenal ARF is the second most common cause of ARF in the elderly, accounting for nearly one-third of all hospitalized cases. Common causes can be grouped into true volume depletion (e.g. decreased fluid intake), decreased effective blood volume (e.g. systemic vasodilation) and haemodynamic (e.g. renal artery stenosis, NSAID use). Acute tubular necrosis (ATN) is the most common cause of intrinsic ARF and is responsible for over 50% of ARF in hospitalized patients, and up to 76% of cases in patients in intensive care units. ATN usually occurs after an acute ischaemic or toxic event. The pathogenesis of ATN involves an interplay of processes that include endothelial injury, microvascular flow disruption, tubular hypoxia, dysfunction and apoptosis, tubular obstruction and trans-tubular back-leak. Vasculitis causing ARF should not be missed as this condition is potentially life threatening. The likelihood of a postrenal cause for ARF increases with age. Benign prostatic hypertrophy, prostatic carcinoma and pelvic malignancies are all important causes. Early identification of ARF secondary to obstruction with renal imaging is essential, and complete or partial renal recovery usually ensues following relief of the obstruction.A comprehensive medical and drug history and physical examination are all invaluable. Particular attention should be paid to the fluid status of the patient (skin turgor, jugular venous pressure, lying and standing blood pressure, urine output). Urinalysis should be performed to detect evidence of proteinuria and haematuria, which will aid diagnosis. Fractional excretion of sodium and urine osmolality may be measured but the widespread use of diuretics in the elderly gives rise to unreliable results. Renal imaging, usually ultrasound scanning, is routinely performed for assessment of renal size and to exclude urinary obstruction. In some cases, renal biopsy is necessary to provide specific diagnostic information. The general principles of managing ARF include treatment of life-threatening features such as shock, respiratory failure, hyperkalaemia, pulmonary oedema, metabolic acidosis and sepsis; stopping and avoiding administration of nephrotoxins; optimization of haemodynamic and fluid status; adjustment of drug dosage appropriate to glomerular filtration rate; early nutritional support; and early referral to nephrologists for diagnosis of ARF cause, timely initiation of dialysis and initiation of specific treatment. The treatment of prerenal and ATN ARF is largely supportive with little evidence of benefit from current pharmacological therapies. Despite advances in critical care medicine and renal replacement therapy, the mortality of ARF has not changed significantly over the last 40 years, with current mortality rates being up to 75%.Drugs & Aging 02/2008; 25(6):455-76. · 2.65 Impact Factor
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ABSTRACT: The aim of this report is to describe the first instance of Wegner's granulomatosis (WG) in a 12-year-old boy at the Children's Medical Center in Tehran University. This report will analyse the variety of clinical manifestations observed in a 12-year-old boy who suffered sinobronchitis for two months before diagnosis. We present a 12-year-old patient who exhibited pathological symptoms of WG prior to diagnosis and treatment. Our patient was monitored for one year after initial therapy through the outpatients department of the Children's Medical Center in Tehran University. One year after treatment, the patient's WG appeared to be in complete remission and he remained entirely well. Although Wegener's granulomatosis is a rare condition in children and can accordingly be confused with more common ailments, accurate and timely diagnosis can be made based on an established record of clinicopathologic features of the disease, and confirmed by biopsy of nasal mucosa.Hong Kong Journal of Paediatrics 01/2008; 13(New series):60-64. · 0.07 Impact Factor