Massive intracerebral hemorrhage associated with Wegener granulomatosis

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<ul><li><p>SHORT COMMUNICATION</p><p>Massive intracerebral hemorrhage associatedwith Wegener granulomatosis</p><p>Mevlut Ceri Levent Ortabozkoyun </p><p>Selman Unverdi Mustafa Kirac Murat Duranay</p><p>Received: 25 June 2010 / Accepted: 8 May 2011 / Published online: 19 May 2011</p><p> Springer-Verlag 2011</p><p>Abstract Wegener granulomatosis (WG) is a necrotizing</p><p>granulomatous vasculitis that predominantly affects air-</p><p>ways and kidneys. But central nervous system involvement</p><p>(711%) is an uncommon. Massive ICH may occur in the</p><p>course of WG, and this serious condition is related with</p><p>high risk of mortality. Therefore, the new treatment strat-</p><p>egies may be considered in addition to classical practices in</p><p>serious organ involvement and recurrent attack. Here, we</p><p>present an adult patient with WG whose disease was</p><p>complicated by a massive intracerebral hemorrhage (ICH),</p><p>which subsequently led to death.</p><p>Keywords Intracerebral hemorrhage Wegenergranulomatosis Vasculitis</p><p>Introduction</p><p>Wegener granulomatosis (WG) is an autoimmune disease</p><p>that predominantly affects airways and kidneys but may</p><p>affect any organ. We present an adult patient with WG</p><p>whose disease was complicated by a massive intracerebral</p><p>hemorrhage (ICH), which subsequently led to death.</p><p>Case report</p><p>A 65-year-old woman was admitted to the hospital with</p><p>fatigue, nausea, vomiting, anorexia, abdominal pain,</p><p>dyspnea, and hemoptysis. She had been receiving hemod-</p><p>ialysis for 1 year because of WG (nodular lung involve-</p><p>ment and diffuse necrotizing cresentric glomerulonephritis</p><p>on renal biopsy had been revealed 1 year before) compli-</p><p>cated by end-stage renal disease. She has regularly taken</p><p>metoprolol 50 mg/day, losartan 50 mg/day, and methyl-</p><p>prednisolone 4 mg/day treatment. On admission, blood</p><p>pressure of 145/95 mmHg, heart rate of 103/min, respira-</p><p>tion rate of 16/min, and respiratory sound were diminished</p><p>and accompanied by fine diffuse rales in both lungs. Initial</p><p>laboratory findings demonstrated an elevated erythrocyte</p><p>sedimentation rate (47 mm/h) and CRP (20.7 mg/dL),</p><p>normocytic anemia (Hb 4.6 g/dL), and mild leukocytosis</p><p>(WBC 13 800/mm3). Blood urea nitrogen and serum cre-</p><p>atinine concentrations were elevated (21.6 mmol/L and</p><p>431 lmol/L, respectively). Serum immunoglobulin G andM were reduced (742 and 27 mg/ml, respectively), but</p><p>serum complement values were within normal limits. The</p><p>p-ANCA was markedly elevated (54.2 U/mL, normal</p><p>\20), while c-ANCA, antinuclear antibody, and anti-DNAantibody were not detected. Viral serologies for HIV,</p><p>hepatitis B and C were negative. Urinalysis showed 3?</p><p>occult blood, and the urinary sediment contained 75</p><p>erythrocyte per high-power field. A chest X-ray showed</p><p>diffuse bilateral pulmonary infiltrate (Fig. 1). We consid-</p><p>ered WG attack because the chest X-ray indicated a clinical</p><p>picture of diffuse alveolar hemorrhage and dropping Hb.</p><p>Steroid pulse therapy was started with methylprednisolone</p><p>500 mg IV daily for 3 days along with intravenous</p><p>cyclophosphamide (500 mg/m2, monthly), and plasma-</p><p>pheresis was simultaneously performed five sessions,</p><p>M. Ceri L. Ortabozkoyun S. Unverdi M. DuranayDepartment of Nephrology, Ankara Education and Research</p><p>Hospital, Ankara, Turkey</p><p>M. Kirac</p><p>Department of Urology, Akay Hospital, Ankara, Turkey</p><p>M. Ceri (&amp;)Safaktepe Mah. Hakan sok. Acar sitesi B blok. No: 24/14,</p><p>Mamak, Ankara, Turkey</p><p>e-mail:</p><p>123</p><p>Rheumatol Int (2012) 32:18131814</p><p>DOI 10.1007/s00296-011-1954-3</p></li><li><p>followed by 60 mg/day oral methylprednisolone. The</p><p>patient improved significantly with clinical resolution of</p><p>respiratory and constitutional symptoms. A repeat chest</p><p>radiograph at the end of 1 week showed resolution of</p><p>pulmonary infiltrate. Her steroid dose tapered down to</p><p>16 mg/day within the next 4 weeks. One month later, the</p><p>patient suddenly developed aphasia and right hemiparesis,</p><p>followed by loss of consciousness and disorientation.</p><p>A head computed tomography scan revealed an extensive</p><p>ICH involving the left temporoparietal and right frontal</p><p>lobes hemorrhage (Fig. 2). She underwent emergency</p><p>craniotomy with evacuation of the hematoma, but the</p><p>patient died on first day.</p><p>Discussion</p><p>WG is a necrotizing granulomatous vasculitis of small- and</p><p>medium-sized vessels that primarily involve the upper and</p><p>lower respiratory tract and kidneys but can affect almost</p><p>any organ, including the central nervous system (CNS).</p><p>Neurologic involvement occurs in 2254% of patients with</p><p>WG. It usually manifests as mononeuritis multiplex and</p><p>polyneuritis; however, CNS involvement (711%) is</p><p>uncommon in WG and ICH as a complication has been</p><p>rarely reported [15].</p><p>The vasculitis of WG may affect arteries, veins, or</p><p>capillaries in the form of capillaritis. Mucosal hemorrhage</p><p>in the lungs and gastrointestinal tract is frequent compli-</p><p>cation of small vessel vasculitides including WG, but much</p><p>less common are intracerebral and intraventricular hem-</p><p>orrhage. ICH may occur as an initial manifestation or latter</p><p>in the disease course despite being on standard immuno-</p><p>suppressive therapy as our patient [2, 6]. This case also</p><p>demonstrates that the development of CNS vasculitis may</p><p>correlate with disease activity in other organs such as</p><p>diffuse pulmonary hemorrhage.</p><p>Traditionally, a combination of steroids and cyclophos-</p><p>phamide has been considered the gold standard regimen of</p><p>CNS vasculitis. Recently, rituximab is a monoclonal anti-</p><p>CD20 antibody that has been used successfully to treat</p><p>refractory WG. In our patient, ICH developed while being on</p><p>this regimen with plasmapheresis and when other manifes-</p><p>tations of her disease seemed to be controlled.</p><p>In summary, we assume that the fatal massive ICH may</p><p>occur in the course of WG and this serious condition is</p><p>related with high risk of mortality. Moreover, the devel-</p><p>opment of CNS vasculitis may be correlated with disease</p><p>activity in other organs. Therefore, the new treatment</p><p>strategies may be considered in addition to classical prac-</p><p>tices in serious organ involvement and recurrent attack</p><p>such as monoclonal anti-CD20 antibody application.</p><p>Acknowledgments The authors have not received funding forresearch on this article.</p><p>References</p><p>1. Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS,</p><p>Travis WD et al (1992) Wegener granulomatosis: an analysis of</p><p>158 patients. Ann Intern Med 116:488498</p><p>2. Memet B, Rudinskaya A, Krebs T, Oelberg D (2005) Wegener</p><p>granulomatosis with massive intracerebral hemorrhage. J Clin</p><p>Rheumatol 11:314318</p><p>3. Kishimoto M, Arakawa KC (2003) A patient with Wegener</p><p>granulomatosis and intraventricular hemorrhage. J Clin Rheumatol</p><p>9:354358</p><p>4. Seror R, Mahr A, Ramanoelina J, Pagnoux C, Cohen P, Guillevin</p><p>L (2006) Central nervous system involvement in Wegener</p><p>granulomatosis. Medicine (Baltimore) 85:5465</p><p>5. Zhang W, Zhou G, Shi Q, Zhang X, Zeng XF, Zhang FC (2009)</p><p>Clinical analysis of nervous system involvement in ANCA-</p><p>associated systemic vasculitides. Clin Exp Rheumatol 27:6569</p><p>6. Granziera C, Michel P, Rossetti AO, Lurati F, Reymond S,</p><p>Bogousslavsky J (2005) Wegener granulomatosis presenting with</p><p>haemorrhagic stroke in a young adult. J Neurol 252:615616</p><p>Fig. 1 Chest X-ray showing diffuse bilateral pulmonary infiltrate dueto alveolar hemorrhage</p><p>Fig. 2 Noncontrast CT brain demonstrating massive left temporopa-rietal (9 9 7 cm) and right frontal (2.5 9 2 cm) lobes hemorrhage</p><p>with mid-line shift</p><p>1814 Rheumatol Int (2012) 32:18131814</p><p>123</p><p>Massive intracerebral hemorrhage associated with Wegener granulomatosisAbstractIntroductionCase reportDiscussionAcknowledgmentsReferences</p></li></ul>