LETTERS TO THE EDITOR
1333
may potentially produce a variety of neurological
complications, some of them secondary to vascular abnormalities and haemorrhagic diathesis. These complications are not commonly recognized and should be
kept in mind when evaluating a patient with 01.
J. NARVAEZ, J. A. NARVAEZ,* C. MAJOS,* M. T. CLAVAGUERA,
J. J. ALEGRE-SANCHO
Department of Rheumatology, Hospital Principes de
Espana and 'Department of Magnetic Resonance
Imaging (IDI), Hospital Duran i Reynals, Ciudad
Sanitaria y Universitaria de Bellvitge, Hospitalet de
Llobregat, Barcelona, Spain
Accepted 24 May 1996
Correspondence to: F. J. Narvaez Garcia, C/ Navas de
Tolosa, n° 312, 3° I1, Barcelona 08027, Spain.
1. Charnas LR, Marini JC. Communicating hydrocephalus, basilar
invagination, and other neurologic features in osteogenesis
imperfecta. Neurology 1993;43:2603-8.
2. Tsipouras P, Barabas G, Matthews WS. Neurologic correlates of
osteogenesis imperfecta. Arch Neurol 1986;43:150-2.
3. Falvo KA, Root L, Bulloug PG. Osteogenesis imperfecta:
clinical evaluation and management. J Bone Joint Surg
1974;56A: 783-93.
4. Sayre MR, Roberge RJ, Evans TC. Nontraumatic hematoma in
a patient with osteogenesis imperfecta. Am J Emerg Med
1987^:298-301.
5. Pozzati E, Poppi M, Gaist G. Acute bilateral extradural
hematomas in a case of osteogenesis imperfecta congenita.
Neurosurgery 1993;13:66-8.
6. Tokoro K, Nakajima F, Yamataki A. Infantile chronic subdural
hematoma with local protrusion of the skull in a case of
osteogenesis imperfecta. Neurosurgery 1988;22:595-8.
7. De Campos JM, Lopez-Ferro MO, Burzaco JA, Boixad6s
JR. Spontaneous carotid-cavernous fistula in osteogenesis
imperfecta. J Neurosurg 1982;56:59O-3.
8. Spiegel BM, Friedman IA, Schwartz SO. Hemorrhagic disease in
osteogenesis imperfecta: study of platelet functional defect. Am
J Med 1951,22:315-21.
9. Evensen SA, Myhre L, Storworken H. Haemostatic studies in
osteogenesis imperfecta. Scand J Hematol 1984;33:77.
10. Morton ME. Excessive bleeding after surgery in osteogenesis
imperfecta. Br J Oral Maxillofac Surg 1987;25:507-ll.
Polyarteritis Nodosa Presenting as
Ischaemic Colitis
SIR—A 37-yr-old woman was admitted because of
fever and weight loss. She had no remarkable medical
history. The 6 months previous to admission, she
presented with fever, severe myalgia and loss of 10 kg.
On admission, she began with bloody diarrhoea. The
physical examination only disclosed mild abdominal
tenderness and a temperature of 39°C. Investigations
were: haemoglobin 10.6 g/dl; leucocytes 11.5 x lC/l;
platelets 475 x 10'/l; ESR 103 mm; CRJP 56 mg/dl
(normal <0.8). ANA, RF, ANCA, hepatitis B virus
and hepatitis C virus serology and cryoglobulins were
negative. The barium enema and colonoscopy showed
signs of colitis around the splenic flexure of the colon
(Fig. 1). The distal descending and sigmoid colon and
the rectum were spared. The radiologist's and
endoscopist's diagnosis was inflammatory bowel
disease (IBD). The microscopic examination of a
colonic biopsy disclosed signs of ischaemic colitis,
FIG. 1.—Barium enema showing segmental colitis. Mucosal ulcers
extend over the splenic flexure and the proximal third of the
descending colon.
with signs of polyarteritis nodosa (PAN) in the
medium-sized arteries.
Treatment included broad-spectrum antibiotics, i.v.
methylprednisolone, 60 mg/day, and three weekly i.v.
pulses of cyclophosphamide of 750, 500 and 500 mg,
respectively. After discharge, a progressive withdrawal
of prednisone was accomplished and, after
3 x monthly pulses of cyclophosphamide, she was
changed to azathioprine. One year after discharge,
PAN has not relapsed.
Although gastrointestinal involvement is frequent in
PAN, the isolated abdominal presentation is very
uncommon and the large bowel is seldom involved
[1]. Since 1975, only five cases of colitis due to PAN
have been published in the English-language literature
(Table I). All patients were symptomatic for a period
ranging from 2 weeks to 1 yr, and four had extraintestinal symptoms. Although colonic involvement
was demonstrated in every case by barium enema or
endoscopy, all underwent surgery.
The presentation of colonic PAN may mimic IBD in
young patients and atherosclerotic ischaemic colitis in
older ones. Thus, any gastrointestinal symptoms which
are preceded by fever, weight loss, myalgia or
arthralgias, should raise the possibility of vasculitis. In
those patients who do not require an urgent intervention, a deep endoscopic biopsy must be taken. This
can give the diagnosis without the need for surgery.
1334
BRITISH JOURNAL OF RHEUMATOLOGY VOL. 35 NO. 12
TABLE I
Summary of reported cases of PAN presenting as ischaemic colitis
Age/onset*
No.
[reference] Year sex
GI
symptoms
Extra-GI
symptoms
Suspected
diagnosis
Findings
in BE or RS
Perforation
Surgery
Outcome
1
[2]
1979
76/4 weeks
M
Bloody
diarrhoea
Myalgia;
weight loss;
fever
Ischaemic
colitis
Colitis in RS
No
Yes
D
2
[3]
3
[4]
4
[5]
5
[6]
1982
52/1 yr
M
None
Myalgia;
weight loss
Colonic
neoplasm
'Apple
core' in BE
No
Yes
S
1984
58/6 weeks
M
Diarrhoea;
abdominal pain
Fever
IBD
Colitis in RS
Yes
Yes
S
1991
28/2 weeks
M
Diarrhoea;
abdominal pain
None
IBD
Colitis in BE
No
Yes
s
1994 48/6 months
M
Abdominal pain; Myalgia;
occult blood +
fever;
weight loss
Polymyositis
Colitis in RS
Yes
Yes
s
6
[PR]
1995
Bloody
diarrhoea;
abdominal pain
PAN
Colitis in BE;
RS;
colonic biopsy
No
No
s
38/6 months
F
Myalgia;
fever;
weight loss
PAN, polyarteritis nodosa; PR, present report; M, male; F, female; GI, gastrointestinal; IBD, inflammatory bowel disease; BE, barium enema;
RS, rectosigmoidoscopy; S, survived; D, died.
In the presence of acute abdominal signs early surgery
is warranted [7]. In their absence, medical treatment
should suffice [8]. In all cases, close observation by a
surgical team must be undertaken. Broad-spectrum
antibiotics should be added, in order to prevent access
of the colonic flora through the disrupted mucosa.
Maintenance monthly i.v. cyclophosphamide pulses
may allow steroid tapering, and also minimize the
side-effects of long-term oral cyclophosphamide, such
as haemorrhagic cystitis, leucopenia and later malignancies [9]. Therapy should be maintained for 1 yr after
remission and then discontinued, according to the low
relapse rate of classic PAN [10].
G. RUIZ-IRASTORZA, M. V. EGURBIDE, C. AGUIRRE
Service of Internal Medicine, Hospital de Cruces,
48903-Bizkaia, Spain
Accepted 17 May 1996
Correspondence
to:
G.
Ruiz-Irastorza,
c/Iparraguirre 22, 7° izda, 48011-Bilbao, Spain.
3.
5.
6.
7.
8.
9.
Guillevin L, Le Thi Huong D, Godeau P, Jais P, Wechsler B.
Clinical findings and prognosis of polyarteritis nodosa and
Churg-Strauss angiitis: a study in 165 patients. Br J Rheumalol
I988;27:258-64.
Wood MK, Read DR, Kraft AR, Barreta PM. A rare cause of
ischemic colitis: polyarteritis nodosa. Dis Colon Rectum
1979;26:428-33.
Meyer GW, Lichtenstein J. Isolated polyarteritis nodosa
affecting the cecum. Dig Dis Sci 1982;27:467-9.
Lee EL, Smith HJ, Miller GL, Burns DIC, Weiner H. Ischemic
pseudomembranous colitis with perforation due to polyarteritis
nodosa. Am J Gastroenterol 1984;79:35-8.
Gullichsen R, Ovaska J, Ekfors T. Polyarteritis nodosa of the
descending colon. Eur J Surg 1991; 157:421-2.
Fort JG, Griffin R, Tahmoush A, Abruzzo JL. Muscle
involvement in polyarteritis nodosa: report of a patient
presenting clinically as polymyositis and review of the literature.
/ Rheumalol 1994;21:945-8.
Lopez LR, Schocket AL, Stanford RE, Claman HN, Kohler PF.
Gastrointestinal involvement in leukocytoclastic vasculitis and
polyarteritis nodosa. / Rheumalol 1980;7:677-84.
Camilleri M, Pusey CD, Chadwick VS. Rees AJ. Gastrointestinal
manifestations of systemic vasculitis. Q J Med 1983;206:141-9.
De Vita S, Neri R, Bombardieri S. Cyclophosphamide pulses in
the treatment of rheumatic diseases: an update. Clin Exp
Rheumalol 1991;9:179-93.
10. Guillevin L, Lhote F. Distinguishing polyarteritis nodosa from
microscopic polyangiitis and implications for treatment. Curr
Opin Rheumatol 1995;7:20-4.
Multiple Cryptococcal Brain Abscesses in
Systemic Lupus Erythematosus
SIR—Cryptococcus neoformans is an important but
overlooked opportunistic infectious agent in an
immunocompromised host [1,2]. Multiple cryptococcal brain abscesses without associated meningitis and
pulmonary lesion are extremely rare, and pose a
diagnostic challenge [3-6]. A 17-yr-old girl with
systemic lupus erythematosus (SLE) was evaluated at
the paediatric rheumatological clinic 2 weeks after the
onset of fever, vomiting and headache. During the past
2 days, there had been three episodes of focal motor
seizure of the right upper limb with postictal paresis.
She had been receiving prednisolone and azathioprine
for 3 yr after the diagnosis of SLE. On admission,
examination of the fundi revealed marked papilloedema in both eyes. The nuchal rigidity and meningeal
sign were absent. Laboratory investigations showed
haemoglobin 12.4 g/dl, platelets 189 x lO^mm3, white
blood cell count 7400/mm3 with 80% neutrophils, 17%
lymphocytes and 3% monocytes. Lymphocyte subset
analysis showed CD3 95%, CD19 2%, CD4 20% and
CD8 66%, with a reversed CD4/CD8 ratio of 0.33.
Serum complement levels were normal (C3c 114 mg/dl
and C4 20.1 mg/dl). Magnetic resonance imaging
(MRI) disclosed a 3 cm diameter ring enhanced mass
in the left posterior frontal parasagittal parenchyma
with irregular enhanced portion in its posterior aspect.
There were several other smaller enhanced masses in
the right temporal, left anterior frontal and parietal
parenchyma (Fig. 1). Cryptococcus neoformans was
isolated from the blood and brain abscess. The patient
was initially treated with amphotericin B (0.6 mg/kg/