Skip to main content

Main menu

  • Home
  • Content
    • First Release
    • Current
    • Archives
    • Collections
    • Audiovisual Rheum
    • COVID-19 and Rheumatology
  • Resources
    • Guide for Authors
    • Submit Manuscript
    • Author Payment
    • Reviewers
    • Advertisers
    • Classified Ads
    • Reprints and Translations
    • Permissions
    • Meetings
    • FAQ
    • Policies
  • Subscribers
    • Subscription Information
    • Purchase Subscription
    • Your Account
    • Terms and Conditions
  • About Us
    • About Us
    • Editorial Board
    • Letter from the Editor
    • Duncan A. Gordon Award
    • GDPR Policy
    • Accessibility
  • Contact Us
  • JRheum Supplements
  • Services

User menu

  • My Cart
  • Log In
  • Log Out

Search

  • Advanced search
The Journal of Rheumatology
  • JRheum Supplements
  • Services
  • My Cart
  • Log In
  • Log Out
The Journal of Rheumatology

Advanced Search

  • Home
  • Content
    • First Release
    • Current
    • Archives
    • Collections
    • Audiovisual Rheum
    • COVID-19 and Rheumatology
  • Resources
    • Guide for Authors
    • Submit Manuscript
    • Author Payment
    • Reviewers
    • Advertisers
    • Classified Ads
    • Reprints and Translations
    • Permissions
    • Meetings
    • FAQ
    • Policies
  • Subscribers
    • Subscription Information
    • Purchase Subscription
    • Your Account
    • Terms and Conditions
  • About Us
    • About Us
    • Editorial Board
    • Letter from the Editor
    • Duncan A. Gordon Award
    • GDPR Policy
    • Accessibility
  • Contact Us
  • Follow jrheum on Twitter
  • Visit jrheum on Facebook
  • Follow jrheum on LinkedIn
  • Follow jrheum on RSS
Research ArticleArticle

Predictors of Damage and Survival in Patients with Wegener’s Granulomatosis: Analysis of 50 Patients

SEVIL KAMALI, BURAK ERER, BAHAR ARTIM-ESEN, AHMET GUL, LALE OCAL, MERAL KONICE, ORHAN ARAL and MURAT INANC
The Journal of Rheumatology February 2010, 37 (2) 374-378; DOI: https://doi.org/10.3899/jrheum.090387
SEVIL KAMALI
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
BURAK ERER
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
BAHAR ARTIM-ESEN
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
AHMET GUL
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
LALE OCAL
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
MERAL KONICE
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
ORHAN ARAL
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
MURAT INANC
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Figures & Data
  • References
  • Info & Metrics
  • PDF
  • eLetters
PreviousNext
Loading

This article has a correction. Please see:

  • Errata - July 01, 2010

Abstract

Objective. To evaluate damage features and impact on survival by Vasculitis Damage Index (VDI) in a cohort of Turkish patients with Wegener’s granulomatosis (WG).

Methods. We enrolled 50 (25 female) patients with WG according to ACR criteria. Birmingham Vasculitis Activity Score (BVAS) and VDI were used to analyze disease activity and damage.

Results. Patients had kidney (82%), upper airway (72%), lung (70%), and nervous system (15%) involvement. Median age at diagnosis was 45 years, time to diagnosis was 3.5 months, and total followup time was 35.5 months. All but one patient was positive for antineutrophil cytoplasmic antibodies (ANCA). Mean final dose and duration of corticosteroid and cyclophosphamide was 15 ± 14 g, 39 ± 33 months and 36 ± 34 g, 21 ± 2 months, respectively. Mean early (e) BVAS were 20.2 ± 7.1 (4–38) (median 21). Mean e-BVAS and e-VDI scores at presentation and final (f)-VDI scores at last visit were 20.2 ± 7.1 (4–38), 3.1 ± 1.7 (median 3) (0–7) and 4.4 ± 2.6 (0–12), consecutively. Disease related damage was prominent in kidneys (50%) and upper airways (27%). Amenorrhea (90%), cataract (28%), and diabetes (24%) were the most frequent treatment related damages. Rapidly progressive glomerulonephritis at presentation (42%) progressed to endstage renal failure in 20%. Relapses occurred in 25% with mean BVAS of 6.5 ± 2.3 (4–11). Survival rate was 77% at 37 months. Deaths occurred early (90% in the first year). f-VDI was high in patients who relapsed (6 ± 3 vs 3.8 ± 2.1, p = 0.03). Logistic regression analysis demonstrated that age at time of diagnosis and e-VDI were lower in survivors with OR = 0.9 (p = 0.06, 95% CI: 0.78–1) and OR = 0.5 (p = 0.04, 95%CI: 0.25–0.98), respectively. In this cohort, e-VDI score of 5 or more was related to death with 98% sensitivity and 56% specificity (p = 0.004) (CI: 0.66–0.95).

Conclusion. Disease related damage outweighed treatment related damage in our cohort of predominantly generalized disease activity. Early damage and older age were found to be predictors of final damage and death.

  • WEGENER’S GRANULOMATOSIS
  • VASCULITIS
  • VASCULITIS DAMAGE INDEX

Wegener’s granulomatosis (WG) is a multisystem disorder characterized by upper and lower airway and kidney involvement due to systemic necrotizing vasculitis and/or granulomatous inflammation1.

Since better survival has now been achieved thanks to immunosuppressives, outcome measures of disease activity, damage, and functional status have become important topics for the systemic vasculitides, including WG2. Damage as a core component of outcome has been described as irreversible tissue or organ loss or dysfunction related to the disease or treatment. The Vasculitis Damage Index (VDI) is the first proposed damage tool for systemic vasculitides including WG3. The VDI has been used in different cohorts to describe damage characteristics, predictors, and relationship with survival4–6.

We describe damage characteristics and the association of damage, relapses, and survival, in a single center WG cohort.

MATERIALS AND METHODS

We included into the study 50 patients with WG who satisfied American College of Rheumatology (ACR) criteria7 and were followed up between 1993 and 2006. Our WG cohort consists of patients diagnosed and followed up in the Rheumatology Division of Istanbul Medical Faculty, a tertiary referral center. Final damage at last followup visit was scored prospectively in all patients who survived. All but 2 patients had systemic involvement.

Cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA)/anti-PR3 was positive in 71% of the patients. ELISA confirmation could not be performed in 11 patients (22%) with positive c-ANCA. Three patients had perinuclear (p)-ANCA and one had p-ANCA/anti-myeloperoxidase positivity. One patient had no ANCA positivity, but presented with necrotizing crescentic glomerulonephritis, sinus involvement, and cavitated lung nodules. All patients who presented with systemic findings and rapidly progressive glomerulonephritis had taken consecutive pulse corticosteroid (CS) treatment (1 g/day for 3 days). High dose CS treatment (1 mg/kg/day) was applied during the remission induction and then tapered after remission was achieved. Patients were treated with intravenous (iv) cyclophosphamide (CYC) (500–1000 mg/3–4 weekly according to body surface, leukocyte count, and creatininemia), or oral CYC (2 mg/kg/day) for remission induction. Oral CYC (1 mg/kg/day) or azathioprine (2 mg/kg/day) was used for maintenance therapy depending on the severity of WG. Uroprotection with mesna was not used routinely. Final dose and duration of CS and CYC were calculated for each patient. Early disease activity and damage were scored from the hospital and outpatient clinic charts, retrospectively. Final damage at last followup visit was scored prospectively in all patients who survived. Early disease activity at presentation was calculated with the new version of BVAS (BVAS 2003) in accordance with the scoring glossary8 (e-BVAS), retrospectively. Number of relapses and mean BVAS scores of relapses were also calculated for each patient. Relapses were defined with the newly developed clinical finding of WG according to BVAS definition. The 10 items of the VDI were evaluated by the same rheumatologist (SK) using each patient’s clinical and laboratory findings at the third month of followup and at the last visit according to the VDI scoring glossary. Early VDI (e-VDI) scores at third month of disease were calculated for all WG patients including nonsurvivors followed up less than 3 months whose disease duration was more than 3 months and who had received immunosuppressive treatment. Final damage scores (f-VDI) at last followup visit were calculated for 40 patients who survived and continued followup. Severe infection data from the hospital charts were noted.

Correlation of e-BVAS and VDI scores was analyzed by Pearson correlation test. Demographic and treatment characteristics, e-BVAS, e-VDI, and f-VDI scores were compared for patients who relapsed or not by Mann-Whitney U test. We compared demographic and treatment characteristics, BVAS, and only e-VDI scores in WG patients who died or survived using the Mann-Whitney U test. Severe damage was defined with e-VDI score predicting mortality for the WG cohort by receiver operating characteristic (ROC) curve analysis. Survival function was evaluated by Kaplan-Meier method. In multivariate analyses, we estimated the prevalence odds ratio (OR) with 95% confidence interval (CI) for the association between survival and demographic, treatment characteristics and BVAS, and early VDI scores using logistic regression.

RESULTS

Demographic findings of 50 WG patients (25 female, 25 male) were as follows: mean age at diagnosis was 44.1 ± 13.2 years (16–74) (median 45), time elapsed from the first symptom to diagnosis was 6.4 ± 8.5 months (1–48) (median 3.5), total duration of followup was 44.9 ± 34.9 months (2–122) (median 35.5). Eighty-two percent of patients had involvement of the kidney, 72% the upper airways, 70% the lungs, 11% the peripheral nervous system, and 4% the central nervous system. Frequency of organ and drug related damage items is shown in Table 1. Gonadal failure could not be examined systematically because of patient unwillingness in males. Osteoporosis (7.5%) was evaluated in only 31 patients. All patients had taken calcium and vitamin D prophylaxis. Malignancy was not observed in any patient. Twenty-one (42%) patients were admitted with acute renal failure and required hemodialysis, of whom 5 (24%) fully recovered and maintained normal creatininemia. Two patients (10%) recovered with high creatininemia and sustained the same levels. End stage renal failure developed in 10 (20%) patients (one underwent cadaveric renal transplantation at the 3rd year of hemodialysis and 4 patients died), of whom 4 died during the acute stage. CYC was used as 3–4 weekly iv pulse therapy in 28% for 12–20 months, daily oral therapy in 20% for 3–79 months, and oral followed by iv therapy in 52% for 54 months. Mean final dose and duration of CS and CYC were as follows: mean final dose of CS (g) 15 ± 14 (3–99) (median 12), mean total duration of CS (mo) 39 ± 33 (1–124) (median 32), mean final dose of CYC (g) 36 ± 34 (0.3–173) (median 29), mean total duration of CYC (months) 21 ± 21 (1–112) (median 19). Mean e-BVAS scores were 20.2 ± 7.1 (4–38) (median 21). Mean e-VDI and f-VDI scores of patients were 3.1 ± 1.7 (median 3) (0–7) and 4.4 ± 2.6 (median 4) (0–12), consecutively. F-VDI was 1 in 4%, 2 in 12%, 3 in 18%, 4 in 10%, 5 in 10%, 6 in 6%, 7 in 6%, 8 in 6%, 9 in 4% and 12 in 2%. Four patients who died in the hospitalization period during the active disease developed sepsis and multiorgan failure under intensive immunosuppressive treatment. One patient on low dose CS developed cytomegalovirus chorioretinitis during the chronic hemodialysis period. One with generalized disease suffered from pulmonary tuberculosis during the remission induction period: both patients were successfully treated with antiviral and antituberculosis treatment concomittant with mild to moderate dose of CS and 3 cycles of high dose iv immunoglobulin.

View this table:
  • View inline
  • View popup
Table 1

. Disease and treatment related damage items of VDI in 50 patients with WG.

Relapses occurred in 10 (25%) patients (7 with 1 relapse, 2 with 2, and 1 with 3 relapses). Relapses were less severe than early disease activity [mean BVAS score for all relapses was 6.5 ± 2.3 (4–11)]. Early and final VDI scores were found to be positively correlated with e-BVAS (p =0.01, r = 0.37 and p = 0.04, r = 0.33, respectively) (Figure 1–2). Comparison of demographic and treatment characteristics, BVAS, and VDI scores in patients according to relapses demonstrated that f-VDI was the only finding that was significantly high in patients who relapsed (6 ± 3 vs 3.8 ± 2.1, p = 0.03) (Figure 3).

Figure 1
  • Download figure
  • Open in new tab
  • Download powerpoint
Figure 1

Correlation of early Birmingham Vasculitis Activity Score (e-BVAS) and Vasculitis Damage Index (e-VDI) scores.

Figure 2
  • Download figure
  • Open in new tab
  • Download powerpoint
Figure 2

Correlation of early Birmingham Vasculitis Activity Score (e-BVAS) and final Vasculitis Damage Index (f-VDI) scores.

Figure 3
  • Download figure
  • Open in new tab
  • Download powerpoint
Figure 3

Final Vasculitis Damage Index (f-VDI) scores in accordance with relapse.

Ten (20%) WG patients died during the acute or chronic phase of renal failure. Four patients (40%) died from active disease and sepsis with multiorgan failure during the hemodialysis period. One patient (10%) with cerebral involvement died from cerebrovascular hemorrhage due to cerebral vasculitis at a very early stage of disease. We were informed through telephone inquiry that 4 patients who were lost to followup at the stage of chronic hemodialysis had died in the first year of this period. One patient who recovered with high creatininemia died suddenly at 2 years’ followup. The survival rate was 77% at 37 months. Older age of disease onset, shorter followup time, and higher e-VDI scores were found in patients who died (Table 2). The doses and duration of immunosuppressives were significantly higher in survivors, as expected.

View this table:
  • View inline
  • View popup
Table 2

Comparison of demographic and treatment characteristics, BVAS, and early VDI scores in patients with WG who survived (Group A) or died (Group B). Values are mean ± SD (median).

e-VDI score of ≥ 5 was related to death with 98% sensitivity and 56% specificity (p = 0.004) (95%CI 0.66–0.95) by ROC curve analysis (Figure 4) . Logistic regression analysis demonstrated that age at time of diagnosis and e-VDI were lower in survivors with OR = 0.9 (p = 0.06, 95%CI 0.78–1) and OR = 0.5 (p = 0.04, 95%CI 0.25–0.98), respectively.

Figure 4
  • Download figure
  • Open in new tab
  • Download powerpoint
Figure 4

ROC curve analysis of early Vasculitis Damage Index (VDI) according to survival.

DISCUSSION

In the era of immunosuppressive treatment, damage has been a major concern with regard to patient outcome in the systemic vasculitides. WG has considerable potential for damage in different tissues and organs that could result in loss of vital organ functions. Early disease activity, relapses, and immunosuppressives are the major determinants of damage. Since 1997 the VDI has been the only validated tool for damage in systemic vasculitides3. Exley, et al stressed the importance of severe damage in the early stages of vasculitis using the VDI scoring system9. In our present study, we investigated the association of damage with early disease activity, relapse, and survival. Our cohort mainly consisted of patients with severe renal involvement, which can be considered a generalized vasculitic form of WG according to the EULAR definition10. Early disease activity has been found to be correlated with both early and final damage scores.

Relapses less severe than the early disease activity were observed in one-fourth of WG patients receiving immunosuppressive treatment during the median followup time of 35 months. No severe renal relapse was observed during followup. Relapse rates differed according to whether the subgroups predominantly presented with granulomatous and/or generalized vasculitic course. Fauci, et al reported a 50% relapse rate in a US National Institutes of Health (NIH) cohort during 21 years of followup. This cohort includes WG patients with mainly upper airway involvement (90%). Renal involvement was reported as 18% at presentation and 77% at the second year of followup11. In the CYCAZAREM study, patients treated with CYC during the maintenance period had 18% flare rate at the first year of followup12. The frequency and severity of relapses might be prevented with longer treatment with CYC (median 19 mo) in our cohort. In different studies, 25% relapse rates were reported in WG patients with renal involvement treated with CYC13–16. Final damage was found to be higher in our patients who relapsed. The association between damage and relapses has been emphasized by others4,9. A severe damage score on VDI predicting mortality was found to be 5, which is consistent with other reports4,9. Older age and significantly higher early damage scores were demonstrated in WG patients who died in comparison to survivors. The shorter duration of time to diagnosis in nonsurvivors, although insignificant, was attributed to more explosive disease presentation.

We compared our patients with different WG cohorts being evaluated for damage using the same tool. Serial VDI scoring to assess increased damage was used in both Norway and US cohorts prospectively4,6. Baseline characteristics including early disease activity and damage and frequencies of major organ involvement of our cohort were mostly comparable to the Norway cohort6. Kidney damage was the most frequent (50%) organ damage in our series. Endstage renal failure as the major determinant of kidney damage developed in 18%, 7%, and 20% of Norway, WGET6, and our cohorts, respectively. Kidney involvement resulting in endstage renal failure was reported in 24% of 37 WG patients during the mean followup time of 6 years in a French cohort17. Prognosis of our patients presenting with dialysis requirement was quite similar to that in other cohorts4,18.

Immunosuppressive treatment was tailored according to the individual needs in this cohort. The treatment of our cohort was mainly based on the NIH treatment protocol for vasculitis. Generalized disease activity at presentation according to the EULAR definition was observed in one-half of our cohort. These patients were treated with CS and CYC for at least one year in accordance with the literature10,16,19–21. Treatment related damage in WG patients was reported to be 42% in the NIH cohort13, similar to rates in the Norway and in our cohorts, but not in the WGET cohort (15%). We observed amenorrhea in almost all (90%) premenopausal women with median age 34 years. Boumpas, et al reported 100% amenorrhea for women older than 31 years after ≥ 15 CYC pulses in a SLE cohort22. We found 10% of hemorrhagic cystitis in patients with symptomatic nonglomerular hematuria who underwent cystoscopy. There were different reported rates (12–29%) in the literature13,23.

We did not observe malignancy in any of our patients during followup. Absence of malignancy could be explained by the relatively short time of followup. The risks of CYC induced acute myleoid leukemia and bladder cancer were found to be high for cumulative CYC doses of > 36 g after 7 years of followup (SIR 9.5, 95% CI 2.6–24) in a recent analysis24. In our cohort only one patient who was exposed to self-administered CYC for 5 years developed myelodysplastic syndrome. CS related damage such as cataract, diabetes, and avascular necrosis was evident in our cohort but could not be compared with others4,6 regarding dose and duration relationships. CS related side effects have been known to be dependent on dose and duration of CS, protective medications, and individual susceptibilities, and could be the reason for distinct results. We explained our findings as the occurrence of various treatment related damage such as diabetes, hypertension, and amenorrhea, in the relatively early stages of disease treated with the high doses of immunosuppressives.

The rate of survival was 80% for mean followup period of 45 months. Survival rates reported from different series were 45%–88% in WG13,17,23,25–28. Time elapsed from the first symptom to diagnosis seems to be longer in survivors although not significant. This might be caused by more indolent disease course in survivors in comparison to nonsurvivors, who had more explosive disease presentation. Early death characterized by higher damage in comparison to survivors was the most striking feature of our cohort. Gottenberg, et al found a 38% mortality rate in patients with WG-related renal disease17. In our patients with WG, acute renal failure was observed in nearly half the patients, which was related to baseline renal damage and correlated with mortality. Renal involvement resulted in death or endstage renal failure in half the patients in accordance with literature11,13,16,18.

We describe a WG cohort having a significant subgroup with generalized disease presentation. There are only a few reports3,4,6 of damage analysis in WG using the VDI. Our report confirms the importance of early damage as a predictor of final damage and survival using a validated damage tool in a group of Turkish patients with WG. Life threatening, disease related early damage was found to be mainly treatment related, reemphasizing the need for effective treatment in the early stages of disease. We found relatively low relapse rates with mild disease activity that might be attributed to longer duration of CYC maintenance therapy. However, we noted the impact of preventing relapses in order to preclude final damage, even though the relapses were less severe than early disease activity. Early menopause in premenopausal women was the most striking adverse event related to CYC. Early aggressive treatment to avoid or reverse loss of organ function in WG patients with severe organ involvement and optimizing maintenance therapy for preventing relapses were important steps for management.

Footnotes

    • Accepted for publication September 18, 2009.

REFERENCES

  1. ↵
    1. Hochberg MC,
    2. Silman AJ,
    3. Smolen JS,
    4. Weinblatt ME,
    5. Weisman MH
    1. Stone JH,
    2. Hoffman GS
    . Wegener’s granulomatosis. In: Hochberg MC, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH editors. Rheumatology. Philadelphia: Elsevier Limited; 20081533–44.
  2. ↵
    1. Seo P,
    2. Luqmani RA,
    3. Flossman O,
    4. Hellmich B,
    5. Herlyn K,
    6. Hoffman GS,
    7. et al.
    The future of damage assessment in vasculitis. J Rheumatol 2007;34:1357–71.
    OpenUrlAbstract/FREE Full Text
  3. ↵
    1. Exley AR,
    2. Bacon PA,
    3. Luqmani RA,
    4. Kitas GD,
    5. Gordon C,
    6. Savage CO,
    7. et al.
    Development and early validation of the Vasculitis Damage Index for the standardized clinical assessment of damage in the systemic vasculitides. Arthritis Rheum 1997;40:371–80.
    OpenUrlCrossRefPubMed
  4. ↵
    1. Koldingsnes W,
    2. Nossent H
    . Predictors of survival and organ damage in Wegener’s granulomatosis. Rheumatology 2002;41:572–81.
    OpenUrlAbstract/FREE Full Text
    1. Kamali S,
    2. Inanc M,
    3. Gul A,
    4. Ocal L,
    5. Polat NG,
    6. Kilicaslan I,
    7. et al.
    Systemic necrotizing vasculitides in Turkey: a comparative analysis of 40 consecutive patients. Rheumatol Int 2005;26:16–20.
    OpenUrlCrossRefPubMed
  5. ↵
    1. Seo P,
    2. Min YI,
    3. Holbrook JT,
    4. Hoffman GS,
    5. Merkel PA,
    6. Spiera R,
    7. et al.,
    8. WGET Research Group
    Damage caused by Wegener’s granulomatosis and its treatment: prospective data from the Wegener’s Granulomatosis Etanercept Trial (WGET). Arthritis Rheum 2005;52:2168–78.
    OpenUrlCrossRefPubMed
  6. ↵
    1. Leavitt RY,
    2. Fauci AS,
    3. Bloch DA,
    4. et al.
    The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum 1990;33:1101–7.
    OpenUrl
  7. ↵
    http://www.vasculitis.org/scoring/BVAS%202003.pdf.
  8. ↵
    1. Exley AR,
    2. Carruthers DM,
    3. Luqmani RA,
    4. Kitas GD,
    5. Gordon C,
    6. Janssen BA,
    7. et al.
    Damage occurs early in systemic vasculitis and is an index of outcome. QJM 1997;90:391–9.
    OpenUrlAbstract/FREE Full Text
  9. ↵
    European therapeutic trials in ANCA-associated systemic vasculitis: disease scoring, consensus regimens and proposed clinical trials. . Clin Exp Immunol 1995;101 Suppl 129–34.
    OpenUrl
  10. ↵
    1. Fauci AS,
    2. Haynes BF,
    3. Katz P,
    4. Wolff SM
    . Wegener’s granulomatosis. Prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 1983;98:76–85.
    OpenUrlCrossRefPubMed
  11. ↵
    1. Jayne D,
    2. Rasmussen N,
    3. Andrassy K,
    4. Bacon P,
    5. Cohen Tervaert JW,
    6. Dadoniene J,
    7. et al.
    For the European vasculitis study group. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med 2003;349:36–44.
    OpenUrlCrossRefPubMed
  12. ↵
    1. Hoffman GS,
    2. Kerr GS,
    3. Leavitt RY,
    4. Hallahan CW,
    5. Lebovics RS,
    6. Travis WD,
    7. et al.
    Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 1992;15;116:488–98.
    OpenUrl
    1. Tervaert JW,
    2. Huitema MG,
    3. Hene RJ,
    4. et al.
    Prevention of relapses in Wegener’s granulomatosis by treatment based antineutrophil antibody titre. Lancet 1990;336:709–11.
    OpenUrlCrossRefPubMed
    1. Jayne DR,
    2. Gaskin G,
    3. Pusey CD,
    4. Lockwood CM
    . ANCA and predicting relapse in systemic vasculitis QJM 1995;88:127–33.
    OpenUrl
  13. ↵
    1. Westman KW,
    2. Bygren PG,
    3. Olsson H,
    4. Rantsam J,
    5. Weslander J
    . Relapse rate, renal survival and cancer morbidity in patients with Wegener’s granulomatosis or microscopic polyangiitis with renal involvement. J Am Soc Nephrol 1998;9:842–52.
    OpenUrlAbstract
  14. ↵
    1. Gottenberg J-E,
    2. Mahr A,
    3. Pagnoux C,
    4. Cohen P,
    5. Mouthon L,
    6. Guillevin L
    for the French Vasculitis Study Group (FVSG). Long-term outcome of 37 patients with Wegener’s granulomatosis with renal involvement. Presse Med 2007;36:771–8.
    OpenUrlCrossRefPubMed
  15. ↵
    1. Mekhail TM,
    2. Hoffmann GS
    . Longterm outcome of Wegener’s granulomatosis in patients with renal disease requiring dialysis. J Rheumatol 2000;27:1237–40.
    OpenUrlPubMed
  16. ↵
    1. Falk RJ,
    2. Hogan S,
    3. Carey TS,
    4. Jennette JC
    . Clinical course of anti-neutrophil cytoplasmic autoantibodies-associated glomerulonephritis and systemic vasculitis. Ann Intern Med 1990;113:656–63.
    OpenUrlPubMed
    1. Hogan S,
    2. Nachman PH,
    3. Wilkman AS,
    4. Jennette JC,
    5. Falk RJ
    . Prognostic markers in patients with antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis. J Am Soc Nephrol 1996;7:23–32.
    OpenUrlAbstract
  17. ↵
    1. Jayne DR
    . Evidence based treatment of systemic vasculitis. Rheumatology 2000;39:585–95.
    OpenUrlFREE Full Text
  18. ↵
    1. Boumpas DT,
    2. Austin HA,
    3. Vaughan EM,
    4. et al.
    Risk of sustained amenorrhea in patients with systemic lupus erythematosus receiving intermittent pulse cyclophosphamide therapy. Ann Intern Med 1993;119:366–9.
    OpenUrlCrossRefPubMed
  19. ↵
    1. Reinhold-Keller E,
    2. Beuge N,
    3. Katza U,
    4. de Groot K,
    5. Rudert H,
    6. Nolle B,
    7. et al.
    An intradisciplinary approach to the care of patients with Wegener’s granulomatosis: long-term outcome in 155 patients. Arthritis Rheum 2000;43:1021–32.
    OpenUrlCrossRefPubMed
  20. ↵
    1. Faurschou M,
    2. Sorensen IJ,
    3. Mellemkjaer L,
    4. Loft AG,
    5. Thomsen BS,
    6. Tvede N,
    7. et al.
    Malignancies in Wegener’s granulomatosis: incidence and relation to cyclophosphamide therapy in a cohort of 293 patients. J Rheumatol 2008;35:100–5.
    OpenUrlAbstract/FREE Full Text
  21. ↵
    1. Anderson G,
    2. Coles E,
    3. Crane M
    . et al. Wegener’s granuloma: A series of 265 British cases seen between 1975 and 1985. A report by a subcommittee of British Thoracic Society Research Committee. QJM 1992;83:427–38.
    OpenUrlAbstract/FREE Full Text
    1. Boki KA,
    2. Dafni U,
    3. Karpouzas GA,
    4. Papasteriades C,
    5. Drosos A,
    6. Moutsopoulos HM
    . Necrotizing vasculitis in Greece. Clinical, immunological and immunogenetic aspects. A study of 66 patients. Br J Rheumatol 1997;36:1059–66.
    OpenUrlAbstract/FREE Full Text
    1. Matteson EL,
    2. Gold KN,
    3. Bloch DA,
    4. Hunder GG
    . Long-term survival of patients with Wegener’s granulomatosis from the American College of Rheumatology Wegener’s granulomatosis classification criteria cohort. Am J Med 1996;101:129–34.
    OpenUrlCrossRefPubMed
  22. ↵
    1. Bligny D,
    2. Mahr A,
    3. Toumelin PL,
    4. Mouthon L,
    5. Guillevin L
    . Predicting mortality in systemic Wegener’s granulomatosis: a survival analysis based on 93 patients. Arthritis Rheum2004;51:83–91.
PreviousNext
Back to top

In this issue

The Journal of Rheumatology
Vol. 37, Issue 2
1 Feb 2010
  • Table of Contents
  • Table of Contents (PDF)
  • Index by Author
  • Editorial Board (PDF)
Print
Download PDF
Article Alerts
Sign In to Email Alerts with your Email Address
Email Article

Thank you for your interest in spreading the word about The Journal of Rheumatology.

NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

Enter multiple addresses on separate lines or separate them with commas.
Predictors of Damage and Survival in Patients with Wegener’s Granulomatosis: Analysis of 50 Patients
(Your Name) has forwarded a page to you from The Journal of Rheumatology
(Your Name) thought you would like to see this page from the The Journal of Rheumatology web site.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
Citation Tools
Predictors of Damage and Survival in Patients with Wegener’s Granulomatosis: Analysis of 50 Patients
SEVIL KAMALI, BURAK ERER, BAHAR ARTIM-ESEN, AHMET GUL, LALE OCAL, MERAL KONICE, ORHAN ARAL, MURAT INANC
The Journal of Rheumatology Feb 2010, 37 (2) 374-378; DOI: 10.3899/jrheum.090387

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero

 Request Permissions

Share
Predictors of Damage and Survival in Patients with Wegener’s Granulomatosis: Analysis of 50 Patients
SEVIL KAMALI, BURAK ERER, BAHAR ARTIM-ESEN, AHMET GUL, LALE OCAL, MERAL KONICE, ORHAN ARAL, MURAT INANC
The Journal of Rheumatology Feb 2010, 37 (2) 374-378; DOI: 10.3899/jrheum.090387
del.icio.us logo Digg logo Reddit logo Twitter logo CiteULike logo Facebook logo Google logo Mendeley logo
  • Tweet Widget
  • Facebook Like
  • Google Plus One
Save to my folders

Jump to section

  • Article
    • Abstract
    • MATERIALS AND METHODS
    • RESULTS
    • DISCUSSION
    • Footnotes
    • REFERENCES
  • Figures & Data
  • References
  • Info & Metrics
  • PDF
  • eLetters

Related Articles

Cited By...

More in this TOC Section

  • Composite Measures for Clinical Trials in Psoriatic Arthritis: Testing Pain and Fatigue Modifications in a UK Multicenter Study
  • GRAPPA Patient Research Partner Network: Update to the GRAPPA 2020 Annual Meeting
  • Instruments Measuring Physical Function for Psoriatic Arthritis Endorsed at GRAPPA 2020 Annual Meeting: Updates of the GRAPPA-OMERACT Working Group
Show more Articles

Similar Articles

Content

  • First Release
  • Current
  • Archives
  • Collections
  • Audiovisual Rheum
  • COVID-19 and Rheumatology

Resources

  • Guide for Authors
  • Submit Manuscript
  • Author Payment
  • Reviewers
  • Advertisers
  • Classified Ads
  • Reprints and Translations
  • Permissions
  • Meetings
  • FAQ
  • Policies

Subscribers

  • Subscription Information
  • Purchase Subscription
  • Your Account
  • Terms and Conditions

More

  • About Us
  • Contact Us
  • My Alerts
  • My Folders
  • RSS Feeds
The Journal of Rheumatology
The content of this site is intended for health care professionals.
Copyright © 2016 by The Journal of Rheumatology Publishing Co. Ltd.
Print ISSN: 0315-162X; Online ISSN: 1499-2752
Powered by HighWire