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The watermelon stomach in scleroderma.

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no other reaction was observed. Two weeks posttreatment
there was no significant clinical improvement. He had early
morning stiffness lasting 120 minutes, an RAI score of 8, and
a VAS pain score of 5.5 cm.
Four weeks after M-T151 treatment he was admitted
to the hospital on an emergency basis because of acute renal
failure. On admission, he was dehydrated and oliguric.
There was no clinical evidence of vasculitis. The hemoglobin
level was 8.1 gdliter, platelet count was 708 X 109/liter, and
ESR was 18 mm/hour. Urine microscopy showed 88 white
cells/mm3 with granular casts; no eosinophils were seen, and
culture was sterile. Serum creatinine was 552 pmoles/liter,
urea 21.7 mmoles/liter, and potassium 6.5 mmoleshter.
Results of liver function tests were normal. Electrocardiogram showed a small P-wave and tenting of the T-wave,
consistent with hyperkalemia. Findings on ultrasound of the
kidneys were normal. Autoantibodies, including anti-DNA,
were again negative. Serum complement levels were normal.
Creatinine clearance was 6 d m i n u t e , and the 24-hour
urinary protein estimation was 0.8 g d i t e r . Peripheral blood
lymphocyte subset analysis showed the following: CD4 1 x
109/liter and CD8 0.34 x 10’Aiter. DTPA (diethylene triamine penta-acetic acid) scan showed perfused, functioning
kidneys of equal size, but selective accumulation of tracer
with increased parenchymal transit time. Renal biopsy
showed interstitial nephritis with pronounced plasma cell
His condition improved with intravenous fluid and
furosemide. Over the next few days, urine output improved
and his serum creatinine level continued to decrease. Three
months later, the serum creatinine level had returned to 112
There are many causes of acquired interstitial nephritis. Among the most common is drug-induced disease,
caused especially by NSAIDs. However, our patient was not
receiving such treatment, and there was no other obvious
cause. Interstitial nephritis can occur in primary SS (4), but
renal manifestations are uncommon in secondary SS. In a
review by Tu et a1 (9,patients with interstitial nephritis had
primary SS with only minor arthritis. Interstitial nephritis is
a histologic diagnosis and, because renal biopsy is not
performed routinely in patients with RA and secondary SS
who have normal renal function, it is possible that mild
subclinical interstitial nephritis may occur in secondary SS.
Our patient experienced chills and rigor during treatment,
which has been attributed to cytokine release by the antiCD4 MAb (6). The latter may have led to an exacerbation of
preexisting subclinical interstitial nephritis related to secondary SS. Other possible pathogenic mechanisms by which
anti-CD4 MAb could induce interstitial nephritis include
formation of immune complexes (7).
Although it is impossible to know the exact role of
anti-CD4 monoclonal antibodies in this patient’s acute renal
failure, the possibility of a link, either causal or by exacerbation of a preexisting subclinical condition, cannot be
excluded. When anti-CD4 MAb is used as an experimental
treatment, investigators should be vigilant in monitoring
possible side effects.
E. H. S. Choy, MB, BCh, MRCP
G. H. Kingsley, BSc (Hons), MB, MRCP
G. S. Panayi, ScD, MD, FRCP
United Medical and Dental Schools
Guy’s Hospital
London, UK
1. Horneff G, Bunnester GR, Emmrich F, Kalden JR: Treatment of
rheumatoid arthritis with an anti-CD4 monoclonal antibody.
Arthritis Rheum 34: 129-140, 1991
Reiter C, Kakavand B, Rieber EP, Schattenkirchner M, Riethmiiller G, Kruger K: Treatment of rheumatoid arthritis with
monoclonal CD4 antibody M-T151: clinical results. and immunopharmacologic effects in an open study including repeated administration. Arthritis Rheum 34525-536, 1991
Ritchie DM, Boyle JA, McInnes JM, Jasani MK, Dalakos TG,
Grieveson P, Buchanan WW: Clinical studies with an articular
index for the assessment of joint tenderness in patients with
rheumatoid arthritis. Q J Med 37:393-406, 1968
Tala1 N, Zisman E, Schur PH: Renal tubular acidosis, glomerulonephritis and immunologic factors in Sjogren’s syndrome.
Arthritis Rheum 11:774-786, 1968
Tu WH, Shearn MA, Lee JC, Hopper J Jr: Interstitial nephritis in
Sjogren’s syndrome. Ann Intern Med 69: 1163-1170, 1968
Horneff G, Krause A, Emmrich F, Kalden JR, Burmester GR:
Elevated levels of circulating tumor necrosis factor-alpha, interferon-gamma and interleukin-2 in systemic reaction induced by
anti-CM therapy in patients with rheumatoid arthritis. Cytokine
3:266-267, 1991
Williams RC Jr: Immune Complexes in Clinical and Experimental Medicine. Cambridge, Harvard University Press, 1980
The watermelon stomach in scleroderma
Gastric antral vascular ectasia, or “watermelon
stomach,” is a rare cause of chronic gastrointestinal bleeding, with characteristic endoscopic and histologic features.
This unusual disorder has rarely been reported in association
with scleroderma.
The patient described here is a 46-year-old woman
with a 4-year history of scleroderma. In April 1992, she was
evaluated by her primary care physician and was found to be
anemic (hemoglobin value 7.0 gm/dl). Esophagogastroduodenoscopy (EGD) (performed elsewhere) showed gastric
ulceration, with no evidence of active bleeding. Treatment
was initiated with omeprazole (Prilosec; Merck, West Point,
PA), 20 mg/day, and hemotransfusion. During the subsequent 4 months, she continued to require 2 units of blood
every 3 weeks. Further investigations, including colonoscopy, radiographic barium study of the upper gastrointestinal tract with small bowel follow-through, radionuclide scan
for sites of bleeding, and mesenteric angiography, revealed
no active gastrointestinal bleeding. At that time, she was
referred to us for further evaluation.
The patient was a nonsmoker, did not drink alcohol,
and did not take nonsteroidal antiinflammatory drugs. Pertinent review of systems revealed only occasional melena and
infrequent blood-tinged stools over the preceding 4 months.
She was taking omeprazole, 20 mglday, and fluoxetine
(Prozac; Dista, Indianapolis, IN), 20 mglday ,for depression.
Physical examination findings were consistent with
scleroderma, with distal as well as proximal skin induration,
spontaneous Raynaud’s phenomenon, and telangiectasia of
the skin of the chest wall. She was hemodynamically stable.
The hemoglobin value was 8.6 gm/dl with a mean corpuscular volume of 76 pm3 (normal 81-95). Prothrombin time and
partial thromboplastin time were normal; hemoquant value
was 33.5 units (normal 0-2).
Another EGD was performed, and this demonstrated
an array of intensely red stripes radiating to the pylorus,
resembling the stripes on a watermelon (Figure 1). There
were no signs of gastric ulceration or active bleeding. Biopsy
samples were taken from this region of the distal gastric
antrum and showed foveolar hyperplasia and superficial
vascular ectasia with organizing fibrin thrombi, consistent
with “watermelon stomach.” Colonoscopy findings were
Treatment consisted of 2 sessions of endoscopic
yttrium-aluminum-garnet (YAG) laser coagulation applied to
the striped lesions over 2 days. There were no complications, and she required no further blood transfusions over 2
months. Following an asymptomatic drop in the hemoglobin
value, another course of YAG laser coagulation was applied.
No further intervention has been needed to date.
The “watermelon stomach” was first described by
Jabbari et a1 in 1984 because of its endoscopic appearance of
stripes on a watermelon (I). Since 1984, approximately 80
cases have been reported in the gastroenterology literature
(2,3), but none in the rheumatology literature. Gostout et a1
reported the largest series, of 45 patients with watermelon
stomach (2). Autoimmune and connective tissue disorders
were present in 28 of the 45 patients (62%), including 6
patients who had scleroderma. Other medical disorders
included cardiovascular disease, chronic renal failure, diabetes, cirrhosis, and cancer.
The pathogenesis of watermelon stomach is currently unknown. It has been proposed that a loosely attached
antral mucosa receives repeated trauma from prolapse
through the pylorus (4). The diagnosis rests upon the endoscopic finding of “watermelon stripes” and the histologic
features of ectatic capillaries with focal thrombosis, dilated
submucosal vessels, and fibromuscular hyperplasia in the
lamina propria (1). EGD with biopsy is the diagnostic test of
choice, since radiologic investigation is uniformly unrevealing.
It is also important to emphasize that even though
active bleeding may not be present at endoscopy, the lesion
is a source of hemorrhage which requires treatment. Several
endoscopic techniques have successfully been used to treat
the chronic bleeding of watermelon stomach, including laser
(2), bipolar electrocautery (5), heater probe (3), and injection
sclerotherapy (6). We currently use the YAG laser; 86% of
our patients require no further transfusions after 1-4 sessions (2).
Although the mechanism of this entity and the nature
of its association with connective tissue diseases are currently unknown, effective treatment can be offered. Rheu-
Figure 1. Endoscopic appearance of the prepyloric region, showing
stripes radiating to the pylorus.
matologists and gastroenterologists should be aware of this
entity when evaluating a patient with scleroderma and occult
gastrointestinal bleeding.
We thank Dr. Christopher Gostoui for photographing the
lesion at endoscopy.
James S. Scolapio, MD
Eric L. Matteson, MD
Mayo Clinic
Rochester, M N
1. Jabbari M, Raeleen C, Lough J, Daly D, Kinnear D, Goresky C:
Gastric antral vascular ectasia: the watermelon stomach. Gastroenterology 87:1165-1170, 1984
Gostout CJ, Viggiano TR, Ahlquist DA, Wang KK, Larson MV,
Balm R The clinical and endoscopic spectrum of the watermelon
stomach. J Clin Gastroenterol 15:256-263, 1992
Petrini JL, Johnston JH: Heat probe treatment for antral vascular
ectasia. Gastrointest Endosc 35:324-328, 1989
Suit PF, Petras RE, Bauer TW, Petrini JL: Gastric antral
vascular ectasia: a histologic and morphometric study of “the
watermelon stomach.” Am J Surg Pathol 11:750-757, 1987
Binmoeller KF, Katon RM: Bipolar electrocoagulation for watermelon stomach. Gastrointest Endosc 36:399402, 1990
Rose JR: Endoscopic injection of alcohol for bleeding from
gastroduodenal vascular anomalies. Br Med J (Clin Res) 295:9394, 1987
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watermelon, scleroderma, stomach
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