SHORT COMMUNICATION

Massive intracerebral hemorrhage associatedwith Wegener granulomatosis

Mevlut Ceri • Levent Ortabozkoyun •

Selman Unverdi • Mustafa Kirac • Murat Duranay

Received: 25 June 2010 / Accepted: 8 May 2011 / Published online: 19 May 2011

� Springer-Verlag 2011

Abstract Wegener granulomatosis (WG) is a necrotizing

granulomatous vasculitis that predominantly affects air-

ways and kidneys. But central nervous system involvement

(7–11%) is an uncommon. Massive ICH may occur in the

course of WG, and this serious condition is related with

high risk of mortality. Therefore, the new treatment strat-

egies may be considered in addition to classical practices in

serious organ involvement and recurrent attack. Here, we

present an adult patient with WG whose disease was

complicated by a massive intracerebral hemorrhage (ICH),

which subsequently led to death.

Keywords Intracerebral hemorrhage � Wegener

granulomatosis � Vasculitis

Introduction

Wegener granulomatosis (WG) is an autoimmune disease

that predominantly affects airways and kidneys but may

affect any organ. We present an adult patient with WG

whose disease was complicated by a massive intracerebral

hemorrhage (ICH), which subsequently led to death.

Case report

A 65-year-old woman was admitted to the hospital with

fatigue, nausea, vomiting, anorexia, abdominal pain,

dyspnea, and hemoptysis. She had been receiving hemod-

ialysis for 1 year because of WG (nodular lung involve-

ment and diffuse necrotizing cresentric glomerulonephritis

on renal biopsy had been revealed 1 year before) compli-

cated by end-stage renal disease. She has regularly taken

metoprolol 50 mg/day, losartan 50 mg/day, and methyl-

prednisolone 4 mg/day treatment. On admission, blood

pressure of 145/95 mmHg, heart rate of 103/min, respira-

tion rate of 16/min, and respiratory sound were diminished

and accompanied by fine diffuse rales in both lungs. Initial

laboratory findings demonstrated an elevated erythrocyte

sedimentation rate (47 mm/h) and CRP (20.7 mg/dL),

normocytic anemia (Hb 4.6 g/dL), and mild leukocytosis

(WBC 13 800/mm3). Blood urea nitrogen and serum cre-

atinine concentrations were elevated (21.6 mmol/L and

431 lmol/L, respectively). Serum immunoglobulin G and

M were reduced (742 and 27 mg/ml, respectively), but

serum complement values were within normal limits. The

p-ANCA was markedly elevated (54.2 U/mL, normal

\20), while c-ANCA, antinuclear antibody, and anti-DNA

antibody were not detected. Viral serologies for HIV,

hepatitis B and C were negative. Urinalysis showed 3?

occult blood, and the urinary sediment contained 75

erythrocyte per high-power field. A chest X-ray showed

diffuse bilateral pulmonary infiltrate (Fig. 1). We consid-

ered WG attack because the chest X-ray indicated a clinical

picture of diffuse alveolar hemorrhage and dropping Hb.

Steroid pulse therapy was started with methylprednisolone

500 mg IV daily for 3 days along with intravenous

cyclophosphamide (500 mg/m2, monthly), and plasma-

pheresis was simultaneously performed five sessions,

M. Ceri � L. Ortabozkoyun � S. Unverdi � M. Duranay

Department of Nephrology, Ankara Education and Research

Hospital, Ankara, Turkey

M. Kirac

Department of Urology, Akay Hospital, Ankara, Turkey

M. Ceri (&)

Safaktepe Mah. Hakan sok. Acar sitesi B blok. No: 24/14,

Mamak, Ankara, Turkey

e-mail: tscer@yahoo.com

123

Rheumatol Int (2012) 32:1813–1814

DOI 10.1007/s00296-011-1954-3

followed by 60 mg/day oral methylprednisolone. The

patient improved significantly with clinical resolution of

respiratory and constitutional symptoms. A repeat chest

radiograph at the end of 1 week showed resolution of

pulmonary infiltrate. Her steroid dose tapered down to

16 mg/day within the next 4 weeks. One month later, the

patient suddenly developed aphasia and right hemiparesis,

followed by loss of consciousness and disorientation.

A head computed tomography scan revealed an extensive

ICH involving the left temporoparietal and right frontal

lobes hemorrhage (Fig. 2). She underwent emergency

craniotomy with evacuation of the hematoma, but the

patient died on first day.

Discussion

WG is a necrotizing granulomatous vasculitis of small- and

medium-sized vessels that primarily involve the upper and

lower respiratory tract and kidneys but can affect almost

any organ, including the central nervous system (CNS).

Neurologic involvement occurs in 22–54% of patients with

WG. It usually manifests as mononeuritis multiplex and

polyneuritis; however, CNS involvement (7–11%) is

uncommon in WG and ICH as a complication has been

rarely reported [1–5].

The vasculitis of WG may affect arteries, veins, or

capillaries in the form of capillaritis. Mucosal hemorrhage

in the lungs and gastrointestinal tract is frequent compli-

cation of small vessel vasculitides including WG, but much

less common are intracerebral and intraventricular hem-

orrhage. ICH may occur as an initial manifestation or latter

in the disease course despite being on standard immuno-

suppressive therapy as our patient [2, 6]. This case also

demonstrates that the development of CNS vasculitis may

correlate with disease activity in other organs such as

diffuse pulmonary hemorrhage.

Traditionally, a combination of steroids and cyclophos-

phamide has been considered the gold standard regimen of

CNS vasculitis. Recently, rituximab is a monoclonal anti-

CD20 antibody that has been used successfully to treat

refractory WG. In our patient, ICH developed while being on

this regimen with plasmapheresis and when other manifes-

tations of her disease seemed to be controlled.

In summary, we assume that the fatal massive ICH may

occur in the course of WG and this serious condition is

related with high risk of mortality. Moreover, the devel-

opment of CNS vasculitis may be correlated with disease

activity in other organs. Therefore, the new treatment

strategies may be considered in addition to classical prac-

tices in serious organ involvement and recurrent attack

such as monoclonal anti-CD20 antibody application.

Acknowledgments The authors have not received funding for

research on this article.

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Fig. 1 Chest X-ray showing diffuse bilateral pulmonary infiltrate due

to alveolar hemorrhageFig. 2 Noncontrast CT brain demonstrating massive left temporopa-

rietal (9 9 7 cm) and right frontal (2.5 9 2 cm) lobes hemorrhage

with mid-line shift

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