Clinical rheumatology, 1995, 14, N ~ 5 551-553

Erosive Polyarthritis as of Wegener's

Presenting Manifestation Granulomatosis

E . J . T E R B O R G , F . J . M . D I S C H * , C . G . M . K A L L E N B E R G * *

Summary We describe a 55-year old female with an erosive symmetric poly- arthritis and negative rheumatoid factor tests. A preliminary diagnosis was made of sero-negative rheumatoid arthritis. About two years after the first signs of ar- thritis, a diagnosis was made ofWegener's granulomatosis, based on bloody nasal discharge with active inflammation of the nasal mucosa with giant cells, the devel- opment of a saddle nose deformity, micro-haematuria and a strongly positive test for c-ANCA. After treatment with prednisone and cyclophosphamide the patient markedly improved. Wegener's granulomatosis has to be considered in a patient presenting with (erosive) (poly)arthritis.

Key words Wegener's Granulomatosis , Erosive Polyarthritis

INTRODUCTION

Wegener's granulomatosis, a necrotizing granuloma- tous vasculitis of unknown origin, has a preditection for the upper and lower respiratory tract and, in most cas- es, the kidneys (1). Musculoskeletal symptoms are (one of) the presenting signs in about 50 % of the cases and in almost 70 % these symptoms occur during the course of the disease (1,2). Musculoskeletal manifestations most frequently observed in Wegener's granulomatosis are myalgias, arthralgias and (poly)arthritis which is, char- acteristically, non-deforming and non-erosive a' 2. As positive tests for rheumatoid factor occur in a high pro- portion of cases in Wegener's granulomatosis, a mis- diagnosis of rheumatoid arthritis has frequently been established (1,3). A few cases have been reported of Wegener's granulomatosis developing in patients with longstanding erosive rheumatoid arthritis (4,5). We here describe a patient with erosive polyarthritis probably as an early manifestation of Wegener's granulomatosis.

PATIENT

A 55-year old female was referred in June 1989 be- cause of arthralgias. Two months later arthritis of the wrists was observed; ESR was 86 mm in the first hour, CRP 55 mg/1, tests for rheumatoid factor were negative. X-rays of the hands and wrists were normal. A prelim-

From the Department of Rheumatology, * Department of Otorhino- laryngology, Sint Antonius Hospital Nieuwegein; ** Department of Clinical Immunology, University Hospital Groningen.

inary diagnosis of sero-negative rheumatoid arthritis was made and the patient was treated with NSAID's. In De- cember 1990 the patient developed symmetric polYarthri- tis of the wrists, knees, MCP's and PIP's of the hands, and MTP's of the feet with morning stiffness lasting for one hour. X-rays of hands and feet showed erosive chang- es (Fig. 1) and, because of persisting disease activity, methotrexate was started in a dosage of 71/2 mg/week.

Since December 1990, in addition, the patient had no- ticed bloody nasal discharge and in May 1991 a saddle nose deformity was observed (Fig. 2) while a test for ANCA was negative. X-rays of the chest were normal. The urinary sediment showed microhaematuria (20-30 erythrocytes per high power field) without casts or pro- teinuria and with normal renal function (serum creati- nine 70/~mol/1). In October 1991 a renal biopsy speci- men proved to be normal. Computerized tomography disclosed mucosal swelling of the sinuses. Nasal endo- scopy showed active mucosal inflammation, large crusts and a large perforation of the nasal septum. A mucosal biopsy specimen of the nose demonstrated active inflam- mation with many granulocytes and some giant cells with- out vasculitis or granulomata. A test for ANCA was strongly positive in a dilution of 1:128, showing a classi- cal cytoplasmic staining (c-ANCA). Tests for rheuma- toid factor remained negative as were serological tests for syphilis and ANA. At that moment the patient was still on MTX therapy. A diagnosis was made of Wegen- er's granulomatosis and treatment was started with pred- nisone 25 mg and cyclophosphamide 50 mg daily. Sub- sequently, arthritis disappeared as did microhaematuria. In addition, in February 1992 c-ANCA became nega-

552 E.J. ter Borg, EJ .M. Disch, C.G.M. Kal lenberg

Figs. la and lb:(a) X-ray of the right wrist showing erosive changes in the distal ulna and os trapezium. (b) X-ray of the feet showing erosive changes in the head of the fifth metatarsal bone.

tive, ESR fell from 74 to 14 mm in the first hour, and CRP fell from 30 to < 5 mg/1. Prednisone was tapered off and subsequently stopped; X-rays at that moment showed partial recovery of erosive changes of the wrists.

DISCUSSION

Though vasculitis was not found, our patient fulfilled the ACR-criteria for the classification of Wegener's gran- ulomatosis (6). The presence of a high titre of c-ANCA, in fact, strongly suggests this diagnosis (7). Arthritis in Wegener's granulomatosis has been believed to be typ- ically non-erosive (2,8). Up to now, only one case has been reported with erosive arthritis as a manifestation of Wegener's granulomatosis (9). It was suggested that the indolent course of Wegener's granulomatosis in that case allowed the development of erosions whereas in most cases of Wegener's granulomatosis the patient pre- sents with an acute and severe illness necessitating ag- gressive treatment as soon as possible (9). Our patient also experienced a rather silent course of Wegener's gran-

ulomatosis and treatment with prednisone and cyclo- phosphamide was given only 26 months after the first signs of arthritis. We believe that our patient suffered from one common disease, i.e. Wegener's granulomato- sis, because of the relatively short interval (16 months) between the development of arthritis and the develop- ment of nasal symptoms. We think a coincidence of both rheumatoid arthritis and Wegener's granulomatosis is unlikely although this possibility never can be excluded. As in the case of Jacobs et al. (9), there seemed to be some healing of erosions after treatment with prednisone and cyclophosphamide.

Saddle nose deformity is a rare but well-known com- plication in rheumatoid arthritis due to (poly)chondri- tis (10). In the present case, however, there were no oth- er signs of chondritis. In addition, the presence of c-AN- CA is strongly suggestive of Wegener's granulomatosis. Our case urges the clinician to remember that polyarthri- tis in Wegener's granulomatosis can mimic early rheu- matoid arthritis.

REFERENCES

1. Hoffman, G.S., Kerr, G.S., Leavitt, R.Y. et al. Wegener's granu- lomatosis: an analysis of 158 patients. Ann Intern Med 1992, 116, 488-498.

2. Nortake, D.T., Weiner, S.R., Bassett L.W. et al. Rheumatic man- ifestations of Wegener's granulomatosis. J Rheumatol 1987, 14, 949-951.

3. Pritchard, M.H. Wegener's granulomatosis presenting as rheuma- toid arthritis (two cases). Proc R Soc Med 1976, 69, 501-504.

4. Sturrock, R.D., Patnesar, R Wegener's granulomatosis occurring in a patient with pre-existing rheumatoid arthritis. Br J Clin Pract 1974, 28, 183-184.

Fig. 2: Saddle nose deformity.

Erosive polyarthritis in Wegener' s granulomatosis 553

5. Sevel, D. Necrogranulomatous keratitis: associated with Wegen- er's granulomatosis and rheumatoid arthritis. Am J Ophtalmol 1967, 63, 250-255.

6. Leavitt, R.Y., Fauci, A.S., Bloch, D.A. et al. The American Col- lege of Rheumatology criteria for the classification of Wegener's granulomatosis. Arthritis Rheum 1990, 33, 1101-1107.

7. Cohen Tervaert, J.W., Woude van der, EJ., Fauci, A.S. et al. Asso- ciation between active Wegener's granulomatosis and anticyto- plasmatic antibodies. Arch Intern Med 1989, 149, 2461-2465.

8. Resnick, D. Polyarteritis and other vasculitides. In: Diagnosis of Bone and Joint Disorders. Eds.: Resnick, D., Niwayama, G., W.B. Saunders Company, Philadelphia, 1988, 1332-1341.

9. Jacobs, R.E, Moore, M., Brower, A. Wegener's granulomatosis presenting with erosive arthritis. Arthritis Rheum 1987, 30, 943- 946.

10. Michet, C.J., McKenna, C.H., Luthra H.S. et al. Relapsing poly- chondritis. Clinical review. Ann Intern Med 1986, 104, 74-75.

Received: 21 September 1994 Revision-accepted: 2 February 1995 Correspondence to: E.J. TER BORG Department of Rheumatology, Sint Antonius Hospital, Koekoekslaan 1, 3435 CM Nieuwegein, THE NETHERLANDS