Lab Med Online.  2015 Jul;5(3):161-164. 10.3343/lmo.2015.5.3.161.

Atypical Wegener's Granulomatosis Accompanied with Rheumatoid Arthritis: A Case Report

• Song SD
• Kim TY

• KMID: 1821785
• DOI: http://doi.org/10.3343/lmo.2015.5.3.161

Abstract

Wegener's granulomatosis (WG) is highly correlated with cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA). Patients with rheumatoid arthritis (RA) rarely contract WG. Although several concurrent cases have been reported overseas, there are no known cases in Korea. Here we report a unique case of RA with atypical WG testing positive for perinuclear ANCA (p-ANCA) and negative for anti-myeloperoxidase (MPO) antibodies. The 62-yr-old female patient presented with multiple joint pain and showed typical blood test results for RA, i.e., an elevated erythrocyte sedimentation rate and C-reactive protein concentration, and positive for rheumatoid factor and anti-cyclic citrullinated peptide antibodies. RA was clear based on a total score of 10 when applying the classification criteria developed by the American College of Rheumatology/European League Against Rheumatism (2010). In an autoimmune target test, speckled and skeleton patterns were observed. In an ANCA test, p-ANCA was observed (titer, 1:2,560), and tests for anti-proteinase 3 (PR3) and anti-MPO antibodies were negative. After admission, multiple nodules were detected on a chest X-ray and a computed tomography scan. We suspected that she had rheumatic nodules or vasculitis and performed an open lung biopsy. We detected necrotic granulomatous vasculitis, classified as WG, thus leading to WG diagnosis. In conclusion, WG was diagnosed in an RA patient who was negative for c-ANCA (negative PR3) and positive for p-ANCA (negative anti-MPO), and this peculiar finding is likely to improve diagnosis in cases of RA with atypical WG.

Keyword

Rheumatoid arthritis; Wegener's granulomatosis; Antineutrophil cytoplasmic antibodies

MeSH Terms

Antibodies
Antibodies, Antineutrophil Cytoplasmic
Arthralgia
Arthritis, Rheumatoid*
Biopsy
Blood Sedimentation
C-Reactive Protein
Classification
Cytoplasm
Diagnosis
Female
Hematologic Tests
Humans
Korea
Lung
Rheumatic Diseases
Rheumatic Nodule
Rheumatoid Factor
Skeleton
Thorax
Vasculitis
Wegener Granulomatosis*
Antibodies
Antibodies, Antineutrophil Cytoplasmic
C-Reactive Protein
Rheumatoid Factor

Reference

1. Godman GC, Churg J. Wegener's granulomatosis: pathology and review of the literature. AMA Arch Pathol. 1954; 58:533–553.

2. Duna GF, Galperin C, Hoffman GS. Wegener's granulomatosis. Rheum Dis Clin North Am. 1995; 21:949–986.

3. 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; 33:1101–1107.

4. Van der Woude FJ, Rasmussen N, Lobatto S, Wiik A, Permin H, van Es LA, et al. Autoantibodies against neutrophils and monocytes: tool for diagnosis and marker of disease activity in Wegener's granulomatosis. Lancet. 1985; 23:425–429.

5. Spoerl D, Pers YM, Jorgensen C. Anti-neutrophil cytoplasmic antibodies in rheumatoid arthritis: two case reports and review of literature. Allergy Asthma Clin Immunol. 2012; 8:19.

6. Szilasi M, Mátyus J, File I, Szücs G, Rákóczi E, Pfliegler G, et al. Association of ANCA-associated vasculitis-rheumatoid arthritis overlap syndrome in four patients: rituximab may be the right choice? Autoimmunity. 2012; 45:304–309.

7. Sturrock RD, Ratnesar P. Wegener's granulomatosis occurring in a patient with pre-existing rheumatoid arthritis. Br J Clin Pract. 1974; 28:183–184.

8. Ohashi H, Itoh M, Ogawa N, Sudo Y, Endo S, Okugawa T, et al. Wegener's granulomatosis in a patient with a rheumatoid arthritis. Intern Med. 1992; 31:1128–1131.

9. Schwarz-Eywill M, Mantaka P, Unger L, Wittek A, Nüsslein H. First-time manifestation of generalized Wegener's granulomatosis despite methotrexate. Br J Rheumatol. 1998; 37:344–345.

10. Chinoy H, McKenna F. Wegener's granulomatosis and rheumatoid arthritis overlap. Rheumatology (Oxford). 2002; 41:588–589.

11. Douglas G, Bird K, Flume P, Silver R, Bolster M. Wegener's granulomatosis in patients with rheumatoid arthritis. J Rheumatol. 2003; 30:2064–2069.

12. Pai S, Panda M. Limited Wegener's granulomatosis presenting as lung nodules in a patient with rheumatoid arthritis: a case report. Cases J. 2008; 1:417.

13. Broshtilova V, Iliev E, Gantcheva M. Etanercept-induced Wegener granulomatosis in a patient with rheumatoid arthritis. Dermatol Ther. 2013; 26(1):73–76.

14. Jagiello P, Aries P, Arning L, Wagenleiter SE, Csernok E, Hellmich B, et al. The PTPN22 620W allele is a risk factor for Wegener's granulomatosis. Arthritis Rheum. 2005; 52:4039–4043.

15. Chung SA, Xie G, Roshandel D, Sherva R, Edberg JC, Kravitz M, et al. Meta-analysis of genetic polymorphisms in granulomatosis with polyangiitis (Wegener's) reveals shared susceptibility loci with rheumatoid arthritis. Arthritis Rheum. 2012; 64:3463–3471.

16. Hemminki K, Li X, Sundquist J, Sundquist K. Familial associations of rheumatoid arthritis with autoimmune diseases and related conditions. Arthritis Rheum. 2009; 60:661–668.

17. Radice A, Bianchi L, Sinico RA. Anti-neutrophil cytoplasmic autoantibodies: methodological aspects and clinical significance in systemic vasculitis. Autoimmun Rev. 2013; 12:487–495.

18. Schmitt WH, van der Woude FJ. Clinical applications of antineutrophil cytoplasmic antibody testing. Curr Opin Rheumatol. 2004; 16:9–17.

19. Pagnoux C, Seror R, Bérezné A, Rouabhia S, Goulvestre C, Guillevin L. Remittent non-destructive polysynovitis in P-ANCA-positive vasculitis patients with anti-CCP antibodies. Joint Bone Spine. 2010; 77:604–607.

20. Mustila A, Korpela M, Mustonen J, Helin H, Huhtala H, Soppi E, et al. Perinuclear antineutrophil cytoplasmic antibody in rheumatoid arthritis: a marker of severe disease with associated nephropathy. Arthritis Rheum. 1997; 40:710–717.

21. Jearn LH, Kim TY. Reevaluation of antineutrophil cytoplasmic antibodies. J Korean Rheum Assoc. 2004; 11:52–56.

22. Hagen EC, Daha MR, Hermans J, Andrassy K, Csernok E, Gaskin G, et al. Diagnostic value of standardized assays for anti-neutrophil cytoplasmic antibodies in idiopathic systemic vasculitis. EC/BCR Project for ANCA Assay Standardization. Kidney Int. 1998; 53:743–753.