Skip to main content
Log in

Wegener's granulomatosis with bilateral necrotizing scleritis, polyarthritis and renal failure efficiently treated with immunosuppressive therapy

  • Case Report
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Summary

A case report of a female patient with Wegener's granulomatosis is presented. After an initial involvement of the upper respiratory tract in the form of a sinusitis, there followed a severe necrotizing bilateral scleritis necessitating the enucleation of the left eye ball. Renal involvement developed as late as 24 months after the onset of the disease and led to renal failure within three months. Throughout the duration of her disease, the patient had joint symptoms in the form of episodes of migratory nondeforming polyarthritis. The administration of corticosteroids alone in daily doses up to 60 mg prednisone failed to control the progression of the disease, while immunosuppressive therapy with cyclophosphamide combined with methylprednisolone pulse therapy and haemodialysis resulted in a marked improvement of renal function and in the subsidence of the ocular and articular symptoms.

This is a preview of subscription content, log in via an institution to check access.

Access this article

We’re sorry, something doesn't seem to be working properly.

Please try refreshing the page. If that doesn't work, please contact support so we can address the problem.

Similar content being viewed by others

References

  1. Curbis, J.J. Recurrence of Wegener's granulomatosis in a cadaver renal allodraft. Transplantation 1983 36, 452–454.

    PubMed  Google Scholar 

  2. Churg, J. et al. Renal disease-classification and atlas of glomerular diseases. Tokyo, Igaku-Shoin, 1982, 188–197.

    Google Scholar 

  3. Fauci, A.S. Granulomatous vasculities: distinct but related. Ann Intern Med 1977, 87, 782–783.

    PubMed  Google Scholar 

  4. Fauci, A.S. et al. Wegener's granulomatosis, prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 1983, 98, 76–85.

    PubMed  Google Scholar 

  5. Fauci, A.S. et.al. Effect of cytotoxic therapy on the renal lesions of Wegener's granulomatosis. In: Proceedings of the Sixth International Congress of Nephrology. Edit, by S. Giovaneti, V. Borominin, D. Amico Basel, S. Karber, 1976, s. 486–491.

  6. Fessel, W.J. The Problem of granulomatosis in rheumatology. In: Rheumatology for Clinicians, Georg Thieme Publ. Stuttgart, 105–115.

  7. Hunder, G.G. Neurotizing vasculities. In: Textbook of Rheumatology, 1st ed. Ed: Kelley W.N. Harris, E.D., Ruddy, S., Hedge, C.B. Philadelphia. W.B. Saunders, 1981, 1145–1224.

    Google Scholar 

  8. Klinge, H. Grenzformen der Periartritis nodosa. Frankf. Z Pathol, 1931, 42, 455–480.

    Google Scholar 

  9. Michels, R.G. Ocular manifestations in connective tissue disorders. In: the Eye in Systemic Disease, 1st ed., Ed. Ryon J.S., Smith, R.E., New York, Grune and Stratton, 1974, 308–309.

    Google Scholar 

  10. Ochodnicky, M. Wegenerova granulomatoza. Vnitrni Lékarstvi, 1983, 29, 12, 1220–1224.

    Google Scholar 

  11. Oliva, D. Wegenerova granulomatoza s neobvykle rychlym prubehem. Vnitrni Lekarstvi, 1984, 30, 8, 809–812.

    PubMed  Google Scholar 

  12. Valchar, J. Wegenerova granulomatoza s neobvyklym klinickym prubehem. Cas Lék Ces, 1975, 112, 6, 177–180.

    Google Scholar 

  13. Watson, P.G. Scleritis and episcleritis. Br J Ophtalmol, 1976, 60, 3, 163–192.

    Google Scholar 

  14. Wegener, F. Uber eine eigenartige rhinogene Granulomatose mit besonderer Beteiligung des Arteriensystems und der Nieren. Beitr Pathol Anat, 1939, 102, 36.

    Google Scholar 

  15. Wegener, F. Uber generalizierte, septische Gefässerkrankungen. Verh Dtsch Ges Pathol, 1936, 29, 202–209.

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Pavelka, K., Dostal, C., Rossmann, P. et al. Wegener's granulomatosis with bilateral necrotizing scleritis, polyarthritis and renal failure efficiently treated with immunosuppressive therapy. Clin Rheumatol 5, 112–117 (1986). https://doi.org/10.1007/BF02030979

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02030979

Key words

Navigation