Percutaneous image-guided biopsy of lung nodules in the assessment of disease activity in Wegener's granulomatosis. (41/627)

OBJECTIVE: In patients with known Wegener's granulomatosis (WG) and persistent chest radiographic abnormalities, assessment for disease activity is often difficult, prompting the need for histological diagnosis to determine appropriate treatment. Here we report the use of automated image-guided core needle biopsy of pulmonary lesions for the assessment of disease activity in WG, rather than for primary diagnosis. METHODS: Image-guided percutaneous core needle biopsy was performed on five occasions in four patients with thoracic WG and persistent radiographic abnormalities of the chest. Clinical features, indication for biopsy, radiographic abnormalities and pathological findings were recorded. RESULTS: Adequate pathological specimens were obtained, allowing exclusion of infection and tumour. Active chronic inflammation with or without vasculitis was demonstrated in each case, indicating the need for further immunosuppressive therapy. A small pneumothorax following biopsy in one case required no treatment. Follow-up chest imaging revealed a reduction in the extent of thoracic disease following therapy in all cases. CONCLUSIONS: The safety and diagnostic accuracy of image-guided core biopsy of thoracic lesions makes it a useful tool in the assessment of disease activity in WG patients with persistent chest radiographic lesions.  (+info)

Amino acid metabolism in Wegener's granulomatosis. (42/627)

The urinary excretion of amino acids and their derivatives in cases of Wegener's granulomatosis have been examined, and the excretion of urinary amino acids in such cases was different from normal and reticulum cell sarcoma. In Wegener's cases, the amino acids which were always found in readily detectable amounts totaled 33; urinary phenylalanine was 4-5 times higher than in normal subjects. From these experimental results, we believe that the observation of amino acid excretion into urine is an important indicator for the diagnosis of Wegener's granulomatosis. In addition, the results of quantitative examination on the urinary amino acid excretion were compared with normal and reticulum cell sarcoma. Comparison of the analytical data on urinary amino acids of the normal and patient groups indicated significant metabolic abnormalities of some amino acids at the earliest stage of Wegener's granulomatosis.  (+info)

Use of methotrexate and glucocorticoids in the treatment of Wegener's granulomatosis. Long-term renal outcome in patients with glomerulonephritis. (43/627)

OBJECTIVE: To determine the long-term renal outcome in patients with Wegener's granulomatosis (WG) and active glomerulonephritis who were treated with methotrexate (MTX) and glucocorticoids. METHODS: An open-label, prospective standardized trial using weekly low-dose MTX and glucocorticoids for the treatment of WG was performed. Forty-two patients were enrolled into the study, of whom 21 had active glomerulonephritis as a disease manifestation. The mean pretreatment level of serum creatinine in the patients with glomerulonephritis was 1.4 mg/dl. The extent of renal function in these patients at the time of their last followup was subsequently examined. RESULTS: Overall, 20 of 21 patients achieved renal remission. At 1 month and 6 months following study entry, the serum creatinine level in all patients either remained stable or improved. The 20 patients have now been followed up for a median time of 76 months (range 20-108 months). Only 2 patients have had a rise of >0.2 mg/dl in their creatinine level from the time of enrollment to the most recent followup examination. Of the remaining 18 patients, 12 have had stable renal function and 6 have had improvement in their creatinine levels by more than 0.2 mg/dl. CONCLUSION: In this study, the use of MTX and prednisone as initial therapy for patients with WG-related glomerulonephritis and a normal or near-normal level of serum creatinine was not associated with a long-term decline in renal function.  (+info)

Cost-effectiveness of prophylaxis against Pneumocystis carinii pneumonia in patients with Wegner's granulomatosis undergoing immunosuppressive therapy. (44/627)

OBJECTIVE: To assess the incremental cost-effectiveness of 3 Pneumocystis carinii pneumonia (PCP) prophylaxis strategies in patients with Wegener's granulomatosis (WG) receiving immunosuppressive therapies: 1) no prophylaxis; 2) trimethoprim/sulfamethoxazole (TMP/SMX) 160 mg/800 mg 3 times a week, which is discontinued if patients experience an adverse drug reaction (ADR); and 3) TMP/SMX 160 mg/800 mg 3 times a week, which is replaced by monthly aerosolized pentamidine (300 mg) if patients experience an ADR. METHODS: A Markov state-transition model was developed to follow a hypothetical cohort of WG patients over their lifetimes starting from the time of initial exposure to the immunosuppressive therapy. The effect of PCP prophylaxis on life expectancy, quality-adjusted life expectancy, average discounted lifetime cost (ADLC), and incremental cost-effectiveness was estimated based on data obtained from a literature review. Direct medical costs were examined from a societal perspective, and costs and benefits were discounted at 3% annually. RESULTS: No prophylaxis resulted in a life expectancy of 13.36 quality-adjusted life years (QALY) at an ADLC of $4,538. In comparison, prophylaxis with TMP/ SMX alone increased the QALY to 13.54 and was cost saving, with an ADLC of $3,304. The addition of pentamidine in patients who had an ADR to TMP/SMX resulted in 13.61 QALY, with an ADLC of $7,428. Compared with TMP/SMX alone, TMP/SMX followed by pentamidine increased the QALY by 0.07 at an incremental cost of $58,037 per QALY. Both TMP/SMX alone and TMP/SMX followed by pentamidine prophylaxis strategies dominated the no prophylaxis strategy until the incidence of PCP fell below 0.2% and 2.25%, respectively. Institution of pentamidine therapy for patients with a TMP/SMX ADR increased quality-adjusted life expectancy compared with that with TMP/ SMX alone until the incidence of PCP rose above 7.5%. CONCLUSION: Prophylaxis using TMP/SMX alone increased life expectancy and reduced cost for patients with WG receiving immunosuppressive therapy. Replacing TMP/SMX with monthly aerosolized pentamidine in cases of ADR further increased life expectancy, although at an increased cost.  (+info)

An unusual case of Wegener's granulomatosis. (45/627)

A 35-year-old Malay man underwent treatment for uveitis of the right eye in 1992 but developed marked visual impairment in the affected eye after he failed to attend follow-up. Two years later, he complained of difficulty swallowing and was found to have left sided X and XI cranial nerve palsies. Chest radiograph showed a cavitating lesion in the lower zone of the right lung field. Inflammation and perforation of the nasal septum was found on examination of his upper respiratory tract. Punch biopsies taken from that area showed chronic inflammatory change and necrotizing vasculities. The patient was diagnosed as having Wegener's granulomatosis and made a very good recovery with immunosuppressive therapy.  (+info)

Radiolabeled leucocyte imaging in diffuse granulomatous involvement of the meninges in Wegener's granulomatosis: scintigraphic findings and their role in monitoring treatment response to specific immunotherapy (humanized monoclonal antilymphocyte antibodies). (46/627)

BACKGROUND AND PURPOSE: Diffuse involvement of the meninges by remote granulomas in Wegener's granulomatosis is rare. This study reports the radiolabeled leucocyte imaging findings in five such patients. The diagnosis was made by MR imaging in five patients and confirmed in four by findings at meningeal biopsy. The potential role of serial radiolabeled leucocyte examinations in assessing treatment response is discussed. METHODS: Three of the five patients underwent whole-body planar 111In-labeled leucocyte imaging. Two of these patients had serial imaging and one had, in addition, a 99mTc-HMPAO-labeled leucocyte single-photon emission CT brain examination. Two of the five patients had whole-body planar 99mTc-HMPAO-labeled leucocyte imaging. Of these, one patient had serial imaging. The radiolabeled leucocyte imaging findings were correlated with MR findings and with the patients' clinical course. RESULTS: In four patients there was a midline linear area of increased tracer uptake in the brain, and in one of these, tracer uptake also extended laterally over the brain convexity. In two patients linear tracer uptake extended in an inferolateral direction from the midline. These abnormalities correlated with meningeal thickening in the falx, over the brain convexity, and in the tentorium cerebelli on MR images. Serial imaging in three patients revealed a reduction or disappearance in tracer uptake after treatment with anti-CD52, which correlated with clinical improvement. CONCLUSION: In patients with Wegener's granulomatosis, abnormal uptake corresponding to meningeal thickening can be seen on planar radiolabeled leucocyte images. Leucocyte imaging may be useful for monitoring treatment response.  (+info)

Granulomatous hypophysitis due to Wegener's granulomatosis. (47/627)

We describe the MR image findings in a case of granulomatous hypophysitis due to Wegner's granulomatosis. A high index suspicion of hypophysitis based on imaging findings allowed successful medical management and helped avoid surgery. The MR imaging features included a thickened stalk, a diffusely and uniformly enlarged gland, a normal size or minimally enlarged sella, and enhancement of the optic chiasm.  (+info)

Pregnancy in patients with Wegener's granulomatosis: report of five cases in three women. (48/627)

Five cases of pregnancy occurring in three women with previously diagnosed Wegener's granulomatosis are described. The disease was diffuse in one case and localised in the other. Initial treatment consisted of a combination of corticosteroids and intravenous cyclophosphamide in two women, and methotrexate in one. Four pregnancies ended in live births despite pre-eclampsia in two cases. One therapeutic abortion was induced because of encephalocele. Comparable reported cases were reviewed to examine the implications of immunosuppressive treatment on the fetus. A relapse occurred during pregnancy in 40% of the cases, but in 25% if only pregnancies beginning during inactive disease were taken into account. No other indicator for maternal and fetal outcome was obvious. Pregnancy should be planned after complete disappearance of disease activity. In the case of a relapse a combination of immunosuppressive drugs and corticosteroids should be chosen rather than corticosteroids alone because the outcome of pregnancy is poor in cases of undertreatment. Prematurity remains common.  (+info)