THE AMERICAN JOURNAL OF GASTROENTEROLOGY
1, 2000
© 2000 by Am. Coll. of Gastroenterology
Published by Elsevier Science Inc.
BRIEF CASE REPORTS
Isolated Abdominal Vasculitis
as an Atypical Presentation
of Wegener’s Granulomatosis
Ya Ju Chang, M.D., and Leslie Dubin Kerr, M.D.
Division of Rheumatology, Department of Medicine, Mount
Sinai Medical Center, New York, New York
ABSTRACT
Although current classifications characterize vasculitic syn-
dromes based upon the size of the vessels involved, the
histopathology, and the presence or absence of antineutro-
phil cytoplasmatic antibodies ANCA (1–3), those occa-
sional patients with vasculitis whose features are not typical
may evade diagnosis and effective treatment. We report one
such patient who presented with bilateral refractory uveitis
and abdominal angina who had a positive C-ANCA. Be-
cause of his atypical presentation, this patient’s disease
progressed over 8 yr despite an extensive gastrointestinal
evaluation, before a diagnosis of vasculitis was established
angiographically, and immunosuppressive therapy was
begun. (Am J Gastroenterol 2000;95:297–298. © 2000 by
Am. Coll. of Gastroenterology)
CASE REPORT
This 47-yr-old man presented with an 8-yr history of bilat-
eral refractory uveitis. He was treated with topical steroids
with some suppression of symptoms; however, he had a
flare of uveitis every time he tried to decrease the number of
steroid eye drops. He developed the insidious onset of
postprandial back pain followed by frontal crampy abdom-
inal pain lasting approximately 20 min to 1 h. He then
started to lose weight. The pain gradually intensified, be-
coming severe, diffuse and incapacitating, making it impos-
sible to eat. He lost ⬎100 pounds over 8 yr. Over the years,
he underwent an evaluation consisting of abdominal CT
scan that was negative, a colonoscopy that showed diver-
ticulosis without inflammatory change, and a magnetic res-
onance imaging plus MRA, both of which were normal. He
was treated with 2 wk of ciprofloxacin without improve-
ment. Two years before presentation, bilateral Achilles ten-
dinitis and left shoulder pain developed. Both resolved with
the use of NSAIDs. He continued to experience recurrent
episodes of this incapacitating abdominal pain, which was
triggered by eating. In the 6 months before presentation, he
developed the new onset of hypertension. He denied a
history of conjunctivitis, oral or genital ulcers, skin rash, any
neurological symptoms, muscle weakness, sinusitis, hemop-
tysis, or renal disease. His medications consisted of topical
steroid eye drops, losartan, loperamide, and multivitamins.
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ISSN 0002-9270/00/$20.00
PII S0002-9270(99)00614-0
His exam upon presentation to us was pertinent for a
2-pound weight loss, normal blood pressure of 140/80 mm
Hg plus a completely unremarkable HEENT, cardiac, pul-
monary, abdominal, and neurological examination. No vas-
culitic skin lesions were seen. Chest x-ray showed no evi-
dence of infiltrates, nodules or cavities. Laboratory values
were: white blood cells 10,700 ⫻ 1,000/ml, hemoglobin
13.8 g/100 ml, hematocrit 40.3%, platelet 331,000/mm3,
ANA, RPR, and Lyme titer were negative. The C-ANCA
was positive at 1:160, and the P-ANCA was negative. The
ESR was 60 mm/h.
The constellation of chronic uveitis, intermittent muscu-
loskeletal inflammation, accelerating postprandial abdomi-
nal pain, accompanied by weight loss of ⬎100 pounds over
8 yr, with new onset of HTN and positive C-ANCA, sug-
gested possible underlying necrotizing vasculitis. Because
no tissue was available for biopsy he underwent mesenteric
and renal angiography. The angiogram showed occlusions
of several jejunal and ileal artery branches at their respective
origins from the superior mesenteric artery, and a 2 cm-long
occlusion of the main trunk of the distal superior mesenteric
artery. Two areas of focal narrowing within proximal por-
tions of splenic artery and diffuse narrowing of several distal
branches of right renal artery were also seen, as well as
small aneurysms within renal vessels. These findings were
diagnostic of abdominal vasculitis (Fig. 1).
The patient was admitted to the hospital for bowel rest,
total parenteral nutrition, and intravenous immunosuppres-
sive therapy consisting of methylprednisolone 48 mg daily
and cyclophosphamide 100 mg daily. Upon discharge, his
ability to eat without pain increased. The steroids were
gradually tapered and cyclophosphamide was increased to
125 mg p.o. q.d. with stabilization of his weight and total
resolution of the uveitis. After 1 yr of remission, he is
currently maintained on 4 mg of oral methylprednisolone
and oral methotrexate.
DISCUSSION
This case demonstrates a constellation of features overlap-
ping the diagnostic criteria for both Wegener’s granuloma-
tosis (WG) and polyarteritis nodosa (PAN). Findings sup-
porting the diagnosis of WG are positive C-ANCA with
sensitivity and specificity of 50 –100% and 90 –97%, respec-
tively, and persistent uveitis (4). However, because more
common features of WG such as airway involvement, glo-
merulonephritis, and pulmonary involvement were not seen
in this patient, this may have contributed to the delay in
diagnosis.
Features of this case consistent with PAN are weight loss
of ⬎4 kg secondary to mesenteric ischemia, new onset of
HTN, and transient elevation of BUN/CRE in conjunction
298 Brief Case Reports
Figure 1. (1) Occlusion of multiple ileal and jejunal branches at
origin of superior mesenteric artery. (2) Occlusion of ileocolic
artery with reconstitution via collateral. (3) Right colic artery
occlusion.
with positive celiac and renal angiograms, per the American
College of Rheumatology (ACR) criteria for the diagnosis
of PAN, with a sensitivity and specificity of 82% and 86%
respectively (5). However the presence of uveitis and the
positive C-ANCA make this case atypical of PAN and most
consistent with WG (4, 5).
Based upon the above clinical features, the positive se-
rology for C-ANCA plus angiographic findings, we believe
that this patient has an atypical presentation of WG. The
most important feature of this case is the morbidity experi-
enced by this patient as a consequence of his unusual,
indolent overlapping symptoms, which delayed his diagno-
sis of vasculitis and the appropriate treatment, which ulti-
mately was lifesaving.
This case serves as a reminder to remain vigilant to the
possibility of systemic vasculitis despite the atypical con-
stellation of the clinical and serological features.
Reprint requests and correspondence: Ya Ju Chang, M.D.,
Division of Rheumatology, Box 1244, Mount Sinai Medical Cen-
ter, 1 Gustave L. Levy Place, New York, NY 10029-6574.
Received Aug. 4, 1998; accepted Jan. 11, 1999.
REFERENCES
1. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of
systemic vasculitides: Proposal of an international consensus
conference. Arth Rheum 1994;37:187–92.
2. Gross LR, Antineutrophil cytoplasmatic autoantibody testing
in vasculitides. Rheum Dis Clin North Am 1995;21:987–1011.
3. Lie JT. Illustrated histopathologic classification criteria for
selected vasculitis syndromes. Arth Rheum 1990;33:1088 –93.
AJG – Vol. 95, No. 1, 2000
4. Duna GF, Galperin C, Hoffman G. Wegener’s granulomatosis.
Rheuma Dis Clin North Am 1995;21:949 – 85.
5. Lhote F, Guillevin L. Polyarteritis nodosa microscopic poly-
angiitis, and Churg-Strauss syndrome. Rheum Dis Clin North
Am 1995;21:911– 47.
Precipitation of Iron Overload and
Hereditary Hemochromatosis After
Successful Treatment of Celiac Disease
Michael A. Heneghan, M.D., Kenneth M. Feeley, M.D.,
Fiona M. Stevens, M.D., Malcolm P. G. Little, M.D., and
Ciaran F. McCarthy, M.D.
Departments of Medicine and Pathology, Clinical Science
Institute, University College Hospital, Galway, Ireland
INTRODUCTION
Hereditary hemochromatosis (HH) is inherited as an auto-
somal recessive trait. It is characterized by increased ab-
sorption of dietary iron, resulting in excess iron deposition
in the parenchymal cells of the liver, heart, and certain
endocrine glands. Premature death can occur from compli-
cations of chronic liver disease, hepatocellular carcinoma,
and heart failure, whereas considerable morbidity is as-
sociated with nonspecific constitutional symptoms and
malaise. It is estimated that up to 5 persons per 1000 of
European descent are homozygous for the condition (1,
2). It is speculated that intact mucosal epithelium of the
proximal small bowel may be critical for the manifesta-
tion of the condition.
CASE REPORT
In February 1986, a 61-yr-old woman presented with weight
loss of 8 kg and malaise. She had a history of anemia for 30
yr and was treated with intermittent oral iron. A nonsmoker
and nondrinker, her family history was noncontributory.
Examination revealed a woman of 45 kg with clubbing but
an otherwise normal examination. Hemoglobin was 11.0
g/dl (normal range [NR] 12–16 g/dl), mean corpuscular
volume 94.9 fL (NR 77–91 fL), platelets 517 ⫻ 109/L (NR
160 – 440 ⫻ 109/L) with Howell Jolly bodies on blood film.
Vitamin B12 and folate levels were normal. Serum iron was
11 (NR 11–26), iron binding capacity 39 ␮mol/L (NR
45–72 ␮mol/L), and serum ferritin 462 ng/ml (NR 10 –200
ng/ml). Celiac disease was confirmed on duodenal biopsy by
the presence of a flat mucosa, increased intraepithelial lym-
phocytes, and chronic inflammatory cells in the lamina
propria. Serum alkaline phosphatase was 167 u/L (NR
30 –115 u/L), with other liver function tests normal. The
patient was discharged from the hospital after 3 wk on a
gluten-free diet (GFD).
By November 1986, she was pigmented. Tests for Addi-