Rheumatol Int (2010) 30:1377–1379

DOI 10.1007/s00296-009-1074-5

CASE REPORT

Recurrent anterior uveitis in Henoch Schonlein’s vasculitis

Burak Erer · Sevil Kamali · Kursat Cingu · Isin Kilicaslan · Ahmet Gul · Murat Inanc · Orhan Aral · Ilknur Tugal-Tutkun

Received: 13 February 2009 / Accepted: 12 July 2009 / Published online: 29 July 2009© Springer-Verlag 2009

Abstract Uveitis is an important component of manyrheumatic diseases. The main causes of recurrent uveitis areseronegative spondylarthropathies and Behçet’s disease.We describe a rare case of Henoch Schönlein vasculitis(HSV) along with multiple recurrences of acute anterioruveitis. In cases of skin rash and recurrent anterior uveitis,HSV should be considered in the diVerential diagnosis.

Keywords Anterior uveitis · Henoch Schonlein’s vasculitis

Case history

A 39-year-old man was referred to our outpatient clinicwith remitting arthralgia in bilateral knees, metacarpopha-

langeal, proximal interphalangeal joints and hips, low backpain, evanescent skin rash and bilateral anterior uveitis.There were no antecedent drug treatments or obvious infec-tion before the occurrence of Wrst symptoms.

He had a history of nonitching maculopapular skin rashin trunk and face, polyarthralgia, acute unilateral epididy-mitis and bilateral anterior uveitis that had recurred threetimes in 5 months before admission. He had been treatedwith topical corticosteroids, nonsteroid antiinXammatorydrugs (NSAID) and antihistaminics without any speciWcdiagnosis. Uveitis and epididymitis had disappeared,although rash and polyarthralgia had remitted several timesunder this treatment. He had no history and clinical Wndingsof Behçet’s disease (BD), seronegative spondylarthropa-thies (SSpA), connective tissue diseases or systemic necro-tizing vasculitides.

Physical examination revealed only maculopapular rashin trunk. Examination of peripheral and axial joints wasnormal. Visual acuity was 20/20 in his both eyes. Therewas bilateral mild ciliary injection, +1 aqueous cells and afew anterior vitreous cells, but the rest of biomicroscopicexamination was normal in his both eyes. Intraocular pres-sure was 18 mmHg in the right eye and 16 mmHg in the lefteye. Fundoscopic examination was normal bilaterally.

Erythrocyte sedimentation rate, full blood count, serumcreatinine, liver function tests and urinalysis were normal.Hepatitis serology, cryoglobulins, antinuclear antibodies,rheumatoid factor, antineutrophil cytoplasmic antibodieswere found negative and serum immunoglobulin A (IgA)titer was in normal range. He had also negative serology ofHLA B 27. X-ray examinations of chest and involved jointswere normal. Magnetic resonance imaging of sacroiliacjoints was performed to rule out sacroiliitis.

Anterior uveitis responded rapidly to topical corticoste-roids and the skin rash remitted under 5–10 mg/day dose of

B. Erer · S. Kamali · A. Gul · M. Inanc · O. AralDivision of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey

B. ErerDivision of Rheumatology, Umraniye Training and Research Hospital, Istanbul, Turkey

K. Cingu · I. Tugal-TutkunDepartment of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey

I. KilicaslanDepartment of Pathology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey

B. Erer (&)Bayar cd, Emintas konut sitesi A Blok D: 6 Sahrayicedid, Kadikoy, 34734 Istanbul, Turkeye-mail: burakerer@yahoo.com

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prednisolone. The patient refused skin biopsy. After6 months he had skin rash and a sudden onset of photopho-bia and red eye. He had a ciliary injection, corneal endothe-lial dusting, and 3+ cells in the anterior chamber in the righteye. There was no Xare or posterior synechiae. Anterioruveitis again responded rapidly to topical corticosteroidtherapy. No cells in the anterior chamber were found at the10-day follow-up visit.

He had three episodes of anterior uveitis, recurrent skinrash and bilateral hip pain while he was on treatment withprednisolone (5 mg/day) and indomethacin (75 mg/day) forthe next 9 months of follow-up until he consented to a skinbiopsy. He had simultaneously bilateral anterior uveitisepisode once and then a unilateral episode in each eye.Each episode was of sudden onset, with mild ciliary injec-tion and 1+ to 3+ cells in the anterior chamber withoutWbrinous reaction. At each episode, photophobia, ciliaryinjection, and anterior chamber cells resolved within daysafter administration of topical corticosteroids. His labora-tory work-up at the time of skin biopsy revealed a higherythrocyte sedimentation rate (70 mm/h), leucocytosis(10.1 £ 109/mm3) and thrombocytosis (457,000/mm3).Skin biopsy revealed leucocytoclastic vasculitis. Immuno-Xuorescence examination demonstrated the deposition ofIgA and IgG in small vessel walls. Henoch Schönlein vas-culitis (HSV) aVecting skin, joints, eyes and testis wasdiagnosed. Prednisolone was increased to the dose of20 mg/day which controlled his symptoms. His visual acu-ity remained 20/20 and he did not develop any complica-tion in the eye.

He was readmitted with arthralgias and similar skin rashin 24th month of the follow-up. He had no constitutionalsymptoms. He had been on treatment of 5 mg/day dose ofprednisolone and 75 mg/day dose of indomethacin. Labora-tory Wndings were as follows; ESR 70 mm/h, hematocrit33%, leucocyte 11.8 £ 109 mm3 granulocyte (80%), throm-bocyte 473,000/mm3, serum creatinine 1.8 mg/dl. Protein-uria (2.6 g/day), hematuria and granular casts were found inurinalysis. ANA, ANCA testing and cryoglobulins wereboth negative. Serum complement levels were found to bedecreased. Kidney biopsy demonstrated mesangial prolifer-ative glomerulonephritis with some partially crescent for-mations and global sclerosis in 50% of the glomeruli(Fig. 1). Mesangial IgA and C3 deposition were found inimmunoXuorescence examination. High dose of daily pred-nisolone (1 mg/kg per day) and monthly cyclophosphamidepulse (1 g/month) therapy was started immediately. Pred-nisolone tapered to maintenance dose of 5 mg/day after3 months and monthly cyclophosphamide pulse wasswitched to azathioprine after 1 year as a maintenance ther-apy. Although persistence of microscopic hematuria wasfound, no relapse occurred and serum creatinine levelsremained stable in upper limits.

Discussion

Herein, we report an atypical case of HSV along withrecurrent anterior uveitis.

Uveitis is an important component of many rheumaticdiseases. The main causes of recurrent uveitis in Turkey areSSpAs and BD [1, 2]. Our patient was not classiWed asSSpA or BD in the absence of sacroiliitis and recurrent oraland genital ulcerations. HLA-B27 serology was also nega-tive. It has been known that recurrent anterior uveitis mayantecede to spinal symptoms in some patients with SSpA[3], but in our case no sacroiliitis was determined duringthe follow-up period of 4 years. Histopathological Wndingsof small vessel vasculitis with IgA deposition conWrmed thediagnosis of HSV.

Scleritis has been known as a part of eye involvement inIgA nephropathy since 1980 [4]. The patient reportedherein did not have episcleral or scleral hyperemia, but hadonly a mild ciliary injection during ocular episodes. There-fore, we did not consider anterior uveitis secondary to epi-scleritis or scleritis in this case.

In 1985, Vanhaesebrock et al. [5] described a patientwith acute tubulointerstitial nephritis and uveitis. This asso-ciation was called as tubulointerstitial nephritis and uveitis(TINU) syndrome. Dobrin et al. [6] described the Wrst casereport with acute renal failure due to tubulointerstitialnephritis accompanied by uveitis and an inXammatory syn-drome in 1975. The association of anterior uveitis and HSVwas Wrst reported by Yamabe et al. [7] in a patient.Although uveitis has been reported in association withHSV, clinical course and ocular implications have not beenspeciWed in previous reports. The patient reported here hadmultiple recurrences of acute anterior uveitis. However,intraocular inXammation was not severe; it was rapidly

Fig. 1 Mesangial proliferative glomerulonephritis with some par-tially crescent formations and global sclerosis in 50% of the glomeruli

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controlled with topical corticosteroids, and did not lead toocular complications. We did not observe a Wbrinous reac-tion, hypopyon formation, or posterior synechiae which areconsidered as typical features of HLA-B27 acute anterioruveitis [8]. Rapid response to topical corticosteroids withindays also helped to make this distinction.

In summary, when a recurrent anterior uveitis is detected,HSV should be considered in the diVerential diagnosis. Incases of typical or atypical skin rash, histopathological evalu-ation including immunoXuorescence examination should becarried out to establish the diagnosis.

ConXict of interest statement None.

References

1. Azizlerli G, Kose AA, Sarica R et al (2003) Prevalence of Behçet’sdisease in Istanbul, Turkey. Int J Dermatol 42:803–806

2. Onen F, Akar S, Birlik M et al (2008) Prevalence of ankylosingspondylitis and related spondyloarthritides in an urban area ofIzmir, Turkey. J Rheumatol 35:305–309

3. Rothova A, van Veenedaal WG, Linssen A et al (1987) Clinical fea-tures of acute anterior uveitis. Am J Opthalmol 103:137–145

4. Nomoto Y, Sakai H, Endoh M et al (1980) Scleritis and IgAnephropathy. Arch Intern Med 140:783–785

5. Vanhaesebrouck P, Carton D, De Bel C et al (1985) Acute tubulo-interstitial nephritis and uveitis syndrome (TINU syndrome).Nephron 40:418–422

6. Dobrin RS, Vernier RL, Fish AL (1975) Acute eosinophilic intersti-tial nephritis and renal failure with bone marrow-lymph nodegranulomas and anterior uveitis. A new syndrome. Am J Med59:325–333

7. Yamabe H, Ozawa K, Fukushi K et al (1988) IgA nephropathy andHenoch-Schönlein purpura nephritis with anterior uveitis. Nephron50:368–370

8. Tuncer S, Adam YS, Urgancioglu M et al (2005) Clinical featuresand outcomes of HLA-B27-positive and HLA-B27-negative acuteanterior uveitis in a Turkish patient population. Ocul ImmunolInXamm 13:367–373

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