Cantoni, Nathan

Daikeler, Thomas

Hess, Christoph

Kappos, Ludwig

Katan, Mira

Meyer, Peter

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CASE REPORTA confusing patient’s history: small or large vessel vasculitis?Nathan Cantoni Æ Peter Meyer Æ Mira Katan ÆLudwig Kappos Æ Christoph Hess Æ Thomas DaikelerReceived: 1 July 2009 / Accepted: 13 September 2009 / Published online: 23 September 2009 Springer-Verlag 2009Case reportWe report on a 59-year-old patient, referred for suddenvisual loss of both eyes. The patient reported blurred visionand conjunctivitis of the left eye since a few days. Addi-tionally fatigue, weight loss (3 kg), night sweats, myalgia,difficulties in hearing on the right ear, frontal headache andpain while chewing (claudicatio masticatoria) since2 weeks were reported.The initial ophthalmological examination revealed avisus of 0.1 on both sides, conjunctivitis was confirmedand funduscopy revealed ischemic retinal lesions withmultiple peripapillary cotton-wool spots (CWS) (Fig. 1).Routine laboratory studies showed thrombocytosis with505 9 109 per L (normal range 150–450 9 109 per L),an increase of the leukocyte count to 15.9 9 109 per L(normal range 3.5–10.0 9 109 per L), an elevated bloodsedimentation rate (BSR) 80 mm/h (normal range 0–19 mm/h) and a C-reactive protein (CRP) of 152 mg/L(normal range \10.0 mg/L). Cranial MRI was compati-ble with mastoiditis on the right side and otherwisenormal.Due to the history, the clinical and laboratory findingsand the results of the fundoscopy, giant cell arteritis (GCA)was diagnosed according to American College of Rheu-matology (ACR) criteria [1], and a steroid therapy withprednisone 1 mg/kg once daily was initiated. However,biopsy results of the temporal artery were unremarkable.Since mastoiditis and conjunctivitis are not typical findingsin GCA, additional tests, including anti-neutrophilic cyto-plasmatic antibodies (ANCA) were performed, showing ac-ANCA titer of 1:640 (normal \1:20) and an anti-pro-teinase 3 (PR3-ANCA) ELISA value of 94U/mL (normal\2.5 U/mL). A subsequent pulmonary CT scan docu-mented multiple small nodules. In light of these findingsthe diagnosis had to be revised to Wegener’s granuloma-tosis (WG) presenting with conjunctivitis, retinal vasculi-tis, mastoiditis, headache, myalgia, and pulmonarygranuloma [2]. A biopsy of the conjunctiva confirmed achronic granulomatous inflammation consistent with theclinical diagnosis of WG. Therapy was intensified withcyclophosphamide 15 mg/kg i.v. every 3 weeks for a totalof six pulses. The patient improved, ANCA titers fell and afollow-up CT scan 6 months after diagnosis showedcomplete resolution of the pulmonary granuloma. Thepatient is with exception of a residual vision loss (visus of0.7), asymptomatic and currently on azathioprine mainte-nance therapy. The CWS and the retinal edemadisappeared.N. Cantoni (&)Division of Hematology, University Hospital Basel,4 Petersgraben, 4031 Basel, Switzerlande-mail: ncantoni@uhbs.chP. MeyerInstitute of Pathology, University Hospital Basel,Basel, SwitzerlandM. Katan  L. KapposDivision of Neurology, University Hospital Basel,Basel, SwitzerlandC. HessMedical Outpatient Department, University Hospital Basel,Basel, SwitzerlandT. DaikelerDivision of Rheumatology, University Hospital Basel,Basel, Switzerland123Rheumatol Int (2010) 30:1681–1683DOI 10.1007/s00296-009-1144-8CommentThis case nicely demonstrates that the diagnosis of WG canbe puzzling [3]. In our patient GCA was the most obviousdiagnosis. However, the presence of mastoiditis and con-junctivitis as well as the normal histology of the temporalartery were reason to question the diagnosis. A positive PR3ANCA was the clue for diagnosing WG. The patients’ischemic symptoms may have been due to necrotizing vas-culitis of the temporal arteries as it has been previouslyreported [4], or due to vasculitis of smaller vessels supplyingeye and the masseter muscle. Because of the normal histol-ogy of the temporal artery, the latter was more likely the case.Granulomatous conjunctivitis without affection of thesclera as well as ischemic retinal lesions due to arterialocclusions are rare manifestations of WG [5–7]. In onlyone patient with WG reported in the literature arterialocclusion was associated with symptoms of GCA [7].Thus, in our patient a rare retinal manifestation of asmall vessel vasculitis mimicked a large vessel vasculitis,reminding us that WG has to be considered as a differentialdiagnosis in atypical GCA.Fig. 1 Nodular conjunctivitisof the left eye withoutassociating episcleritis/scleritis(a, b). Fundoscopy (c) andfluorescein angiogram (d) withischemic retinal lesion withmultiple peripapillary cotton-wool spots (CWS) on the righteye. Fundoscopy (e) andfluorescein angiogram (f) of theleft eye with retinal edema1682 Rheumatol Int (2010) 30:1681–1683123References1. Hunder GG, Bloch DA, Michel BA et al (1990) The AmericanCollege of Rheumatology 1990 criteria for the classification ofgiant cell arteritis. Arthritis Rheum 33:11222. Leavitt RY, Fauci AS, Bloch DA et al (1990) The AmericanCollege of Rheumatology 1990 criteria for the classification ofWegener’s granulomatosis. Arthritis Rheum 33:11013. Reinhold-Keller E, Beuge N, Latza U et al (2000) An interdis-ciplinary approach to the care of patients with Wegener’sgranulomatosis: long-term outcome in 155 patients. ArthritisRheum 43:10214. Miller NR (2001) Visual manifestations of temporal arteritis.Rheum Dis Clin North Am 27(4):781–7975. Harman LE (1998) Wegener’s granulomatosis (review). SurvOphthalmol 42:458–4806. Toh TY (2006) Wegener’s granulomatosis presenting with pain-less bulbar-conjunctival ulcer and retinal artery occlusion. ClinExp Opthalmol 34:51–537. Lamprecht P et al (2000) Retinal artery occlusion in Wegener’sgranulomatosis. Rheumatology 39:928–929Rheumatol Int (2010) 30:1681–1683 1683123

A confusing patient's history: small or large vessel vasculitis?

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A confusing patient's history: small or large vessel vasculitis?

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