J Rheum Dis.  2014 Oct;21(5):257-260. 10.4078/jrd.2014.21.5.257.

A Case of Relapsing Granulomatosis with Polyangiitis Treated Successfully with Rituximab

• Han JW
• Kang KY
• Ban TH
• Jo IH
• Park SH

• KMID: 2222950
• DOI: http://doi.org/10.4078/jrd.2014.21.5.257

Abstract

Granulomatosis with polyangiitis (GPA) is a rare antineutrophil cytoplasmic antibody associated with systemic disease characterized by granulomas and vasculitis affecting small and medium vessels. Neurological manifestations in GPA are less frequent than classical manifestations, such as lung and kidney involvement, and cranial nerve palsies are much rarer. Cyclophosphamide and glucocorticoids have been conventionally administered as an initial induction immunosuppressive therapy for GPA. However, increasing evidence has demonstrated the efficacy and safety of rituximab, an anti-B cell monoclonal antibody, for the treatment of GPA. Herein, we describe a successful treatment of relapsing GPA with cranial nerve involvement using rituximab in a 56-year-old male patient who was previously treated with cyclophosphamide plus glucocorticoids.

Keyword

Granulomatosis with polyangiitis; Cranial nerve; Rituximab

MeSH Terms

Antibodies, Antineutrophil Cytoplasmic
Cranial Nerve Diseases
Cranial Nerves
Cyclophosphamide
Glucocorticoids
Granuloma
Humans
Kidney
Lung
Male
Middle Aged
Neurologic Manifestations
Vasculitis
Rituximab
Antibodies, Antineutrophil Cytoplasmic
Cyclophosphamide
Glucocorticoids

Figure

• 

  Figure 1. Biopsy specimens of the paranasal sinus showing necrotic granulomatous vasculitis without caseous necrosis (A: Hematoxylin and eosin stain, ×40; B: ×200).

• 

  Figure 2. (A) Contrastenhanced T1-weighted image showing an enhancing nodule at the Rt. petro-clival region, entrance site of the right abducens nerve, and Do-rello's canal, suggesting Wegener's granuloma. (B) Contrast enhanced T1-weighted image showing complete resolution of an enhancing nodule after 8 weeks of treatment with rituximab.

Reference

1. Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992; 116:488–98.

2. Seo P, Stone JH. The antineutrophil cytoplasmic anti-body-associated vasculitides. Am J Med. 2004; 117:39–50.

3. Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, et al. RAVE-ITN Research Group. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010; 363:221–32.

4. Song EJ, Kang JW, Kim JH, Kang JY, Nam EJ, Kang YM. Successful treatment of refractory livedoid vasculitis with rituximab and cyclophosphamide. Korean Journal of Internal Medicine. 2010; 78:527–30.

5. Nagashima T, Maguchi S, Terayama Y, Horimoto M, Nemoto M, Nunomura M, et al. P-ANCA-positive Wegener's granulomatosis presenting with hypertrophic pa-chymeningitis and multiple cranial neuropathies: case report and review of literature. Neuropathology. 2000; 20:23–30.

6. Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum. 1990; 33:1101–7.

7. Holle JU, Gross WL. Neurological involvement in Wegener's granulomatosis. Curr Opin Rheumatol. 2011; 23:7–11.

8. de Groot K, Harper L, Jayne DR, Flores Suarez LF, Gregorini G, Gross WL, et al. EUVAS (European Vasculitis Study Group). Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial. Ann Intern Med. 2009; 150:670–80.

9. Jayne D, Rasmussen N, Andrassy K, Bacon P, Tervaert JW, Dadoniené J, et al. 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.

10. Pagnoux C, Mahr A, Hamidou MA, Boffa JJ, Ruivard M, Ducroix JP, et al. French Vasculitis Study Group. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med. 2008; 359:2790–803.

11. Hiemstra TF, Walsh M, Mahr A, Savage CO, de Groot K, Harper L, et al. European Vasculitis Study Group (EUVAS). Mycophenolate mofetil vs azathioprine for remission maintenance in antineutrophil cytoplasmic anti-body-associated vasculitis: a randomized controlled trial. JAMA. 2010; 304:2381–8.

12. Aouba A, Pagnoux C, Bienvenu B, Mahr A, Guillevin L. Analysis of Wegener's granulomatosis responses to rituximab: current evidence and therapeutic prospects. Clin Rev Allergy Immunol. 2008; 34:65–73.

13. Aries PM, Hellmich B, Voswinkel J, Both M, Nölle B, Holl-Ulrich K, et al. Lack of efficacy of rituximab in Wegener's granulomatosis with refractory granulomatous manifestations. Ann Rheum Dis. 2006; 65:853–8.

14. Rhee EP, Laliberte KA, Niles JL. Rituximab as maintenance therapy for anti-neutrophil cytoplasmic antibody-associated vasculitis. Clin J Am Soc Nephrol. 2010; 5:1394–400.

15. Tesfa D, Ajeganova S, Hägglund H, Sander B, Fadeel B, Hafström I, et al. Late-onset neutropenia following rituximab therapy in rheumatic diseases: association with B lymphocyte depletion and infections. Arthritis Rheum. 2011; 63:2209–14.