•
Pneumatosis Intestinalis in Systemic Lupus Erythematosus 1
•
David Freiman, M.D., HiKon Chon, M.D., and Larissa Bllaniuk, M.D. A benign episode of pneumatosis intestinalis developed in a 54-year old black woman with rapidly progressive systemic lupus erythematosus. This case illustrates that pneumatosis intestinalis in association with the various collagen diseases can have disparate prognostic implications. Intestines. cysts. Lupus erythematosus
INDEX TERMS:
Radiology 118:563-564, September 1975
•
•
is a disorder characterized by gas in the intestinal wall and may be associated with many diseases including collagen-vascular diseases such as scleroderma (2), dermatomyositis (7), and rheumatoid arthritis (8). Several recent reports have stressed a grave prognosis when pneumatosis intestinalis develops in patients with collagen disease (6). We observed a patient with systemic lupus erythematosis (SLE) in whom transient pneumatosis intestinalis of the large intestine developed. A careful review of the world literature revealed no record of pneumatosis associated specifically with SLE. NEUMATOSIS
P
t·~ ,. ., '.•:;.:,' . 'f'
INTESTINALIS
CASE REPORT
Diagnostic Radiology
Pneumatosis intestinalis developed in a 54-year-old black woman following a six-month history of unexplained intermittent fever, skin rash, seizures. and weight loss. These symptoms were unaffected by antibiotics, but diminished with steroid therapy. Initial work-up for collagen disease with multiple lupus erythematosus (LE) preparations, antinuclear antibody (ANA) titers, bone marrow biopsy, and rectal biopsy was nondiagnostic. During hospitalization for continued diagnostic work-up, abdominal radiographs demonstrated gas in the wall of the colon and in the root of the mesentery (Fig. 1). A barium enema examination was negative except for a massive pneumatosis. Physical examination of the abdomen and chest demonstrated no abnormality. Chest radiographs and blood gases were all within normal limits. There was no evidence of emphysema, chronic lung disease, or pneumothorax. After several days. the intramural gas spontaneously disappeared. Five months later, repeated LE preparations and ANA titers became markedly positive and the diagnosis of SLE was made. The disease progressed for another three months with renal and pulmonary complications and the patient died of massive pneumonia and respiratory failure eight months after the episode of pneumatosis intestinalis.
DISCUSSION A review of the literature by Mueller et al. (7) disclosed 20 cases of pneumatosis intestinalis associated with scleroderma, rheumatoid arthritis, and dermatomyositis. The etiology
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Fig. 1. Pneumatosis coli. Barium enema shows extensive gas in the wall of the ascending and transverse colon. Fig. 2. Postevacuation radiograph demonstrates a linear air collection in the splenic flexure and typical bubbles of air in the wall of the ascending colon and in the root of the mesocolon.
1 From
the Department of Radiology. Hospital of the University of Pennsylvania, Philadelphia, Pa. Accepted for publication in April 1975.
563
vb
564
DAVID FREIMAN AND OTHERS
and pathogenesis of pneumatosis intestinalis remain unknown. In a review of the literature of 213 cases of pneumatosis intestinalis by Koss (4). 75% were associated with gastrointestinal lesions. Several possible theories have been proposed for the pathogenesis of pneumatosis including bacterial,nutritional, mechanical (5), and pulmonary (3). The mechanical cause may playa role in collagen diseases due to the altered structural framework of the gastrointestinal tissues; however, there is no direct supporting evidence for this theory. The effect of steroid therapy on the development of pneumatosis in collagen diseases is unclear since most of these patients are receiving steroid therapy. Meihoff et al. (6) and Gompels (1) stress a grave prognosis when pneumatosis intestinalis occurs in the small intestine in patients with collagen disease, specifically scleroderma. Mueller et al. (7) described a case of pneumatosis coli which resolved promptly in a patient with dermatomyositis. These different courses indicate that several factors may he responsible for pneumatosis in collagen disease. Intestinal ischemia which has been reported in SLE (9) would appear to be a likely cause of pneumatosis. Bowel wall air in ischemic disease, however, indicates an extremely grave prognosis. In our patient, there were no clinical findings to indicate ischemia and the pneumatosis resolved spontaneously. This case, therefore, is an example of a benign type of pneumatosis occurring in a patient with rapidly progressive SLE.
September 1975
REFERENCES 1. Gompels BM: Pneumatosis cystoides intestinaUs associated with progressive systemic sclerosis. Br J Radiol 42:701-703. Sep 1969 2. Hughes OTO, Gordon KC, Swann JC, et al: Pneumatosis cystoides intestinalis. Gut 7:553-557, Oct 1966 3. Keyting WS, McCarver RR, Kovarik JL, et al: Pneumatosis intestinalis: a new concept. Ra~iology 76:733-741, May 1961 4. Koss LG: Abdominal gas cysts (pneumatosis cystoides intestinorum hominis). An analysis with a report of a case and a critical review of the literature. Arch PathoI53:523-549, Jun 1952 5. Marshak RH, Blum SO, Eliasoph J: Pneumatosis involving left side of colon. JAMA 161:1626-1628, 25 Aug 1956 6. Meihoff WE, Hirschfield JS, Kern J Jr: Small intestinal scleroderma with malabsorption and pneumatosis cystoides intestinalis. Report of three cases. JAMA 204:854-858,3 Jun 1968 7. Mueller CF, Morehead R, Alter AJ, et al: Pneumatosis intestinalis in collagen disorders. Am J Roentgenol 115:300-305, Jun 1972 8. Seaman WB, Fleming RJ, Baker OH: Pneumatosis intestinaUs of small bowel. Semin RoentgenoI1:234-242, Apr 1966 9. Shapeero LG, Myers A, Oberkircher PE, et al: Acute reversible lupus vasculitis of the gastrointestinal tract. Radiology 112: 569-574, Sep 1974
Department of Radiology Hospital of the University of Pennsylvania 3400 Spruce Street Philadelphia, Pa. 19104
Pneumatosis Intestinalis in Systemic Lupus Erythematosus 1
•
David Freiman, M.D., HiKon Chon, M.D., and Larissa Bllaniuk, M.D. A benign episode of pneumatosis intestinalis developed in a 54-year old black woman with rapidly progressive systemic lupus erythematosus. This case illustrates that pneumatosis intestinalis in association with the various collagen diseases can have disparate prognostic implications. Intestines. cysts. Lupus erythematosus
INDEX TERMS:
Radiology 118:563-564, September 1975
•
•
is a disorder characterized by gas in the intestinal wall and may be associated with many diseases including collagen-vascular diseases such as scleroderma (2), dermatomyositis (7), and rheumatoid arthritis (8). Several recent reports have stressed a grave prognosis when pneumatosis intestinalis develops in patients with collagen disease (6). We observed a patient with systemic lupus erythematosis (SLE) in whom transient pneumatosis intestinalis of the large intestine developed. A careful review of the world literature revealed no record of pneumatosis associated specifically with SLE. NEUMATOSIS
P
t·~ ,. ., '.•:;.:,' . 'f'
INTESTINALIS
CASE REPORT
Diagnostic Radiology
Pneumatosis intestinalis developed in a 54-year-old black woman following a six-month history of unexplained intermittent fever, skin rash, seizures. and weight loss. These symptoms were unaffected by antibiotics, but diminished with steroid therapy. Initial work-up for collagen disease with multiple lupus erythematosus (LE) preparations, antinuclear antibody (ANA) titers, bone marrow biopsy, and rectal biopsy was nondiagnostic. During hospitalization for continued diagnostic work-up, abdominal radiographs demonstrated gas in the wall of the colon and in the root of the mesentery (Fig. 1). A barium enema examination was negative except for a massive pneumatosis. Physical examination of the abdomen and chest demonstrated no abnormality. Chest radiographs and blood gases were all within normal limits. There was no evidence of emphysema, chronic lung disease, or pneumothorax. After several days. the intramural gas spontaneously disappeared. Five months later, repeated LE preparations and ANA titers became markedly positive and the diagnosis of SLE was made. The disease progressed for another three months with renal and pulmonary complications and the patient died of massive pneumonia and respiratory failure eight months after the episode of pneumatosis intestinalis.
DISCUSSION A review of the literature by Mueller et al. (7) disclosed 20 cases of pneumatosis intestinalis associated with scleroderma, rheumatoid arthritis, and dermatomyositis. The etiology
.
;,';·,4.,,"
u "
'.""","."'1. ' ; '.,
,', ,',:.
•...
.~
.'\.,
,
".
#i
'.,
Fig. 1. Pneumatosis coli. Barium enema shows extensive gas in the wall of the ascending and transverse colon. Fig. 2. Postevacuation radiograph demonstrates a linear air collection in the splenic flexure and typical bubbles of air in the wall of the ascending colon and in the root of the mesocolon.
1 From
the Department of Radiology. Hospital of the University of Pennsylvania, Philadelphia, Pa. Accepted for publication in April 1975.
563
vb
564
DAVID FREIMAN AND OTHERS
and pathogenesis of pneumatosis intestinalis remain unknown. In a review of the literature of 213 cases of pneumatosis intestinalis by Koss (4). 75% were associated with gastrointestinal lesions. Several possible theories have been proposed for the pathogenesis of pneumatosis including bacterial,nutritional, mechanical (5), and pulmonary (3). The mechanical cause may playa role in collagen diseases due to the altered structural framework of the gastrointestinal tissues; however, there is no direct supporting evidence for this theory. The effect of steroid therapy on the development of pneumatosis in collagen diseases is unclear since most of these patients are receiving steroid therapy. Meihoff et al. (6) and Gompels (1) stress a grave prognosis when pneumatosis intestinalis occurs in the small intestine in patients with collagen disease, specifically scleroderma. Mueller et al. (7) described a case of pneumatosis coli which resolved promptly in a patient with dermatomyositis. These different courses indicate that several factors may he responsible for pneumatosis in collagen disease. Intestinal ischemia which has been reported in SLE (9) would appear to be a likely cause of pneumatosis. Bowel wall air in ischemic disease, however, indicates an extremely grave prognosis. In our patient, there were no clinical findings to indicate ischemia and the pneumatosis resolved spontaneously. This case, therefore, is an example of a benign type of pneumatosis occurring in a patient with rapidly progressive SLE.
September 1975
REFERENCES 1. Gompels BM: Pneumatosis cystoides intestinaUs associated with progressive systemic sclerosis. Br J Radiol 42:701-703. Sep 1969 2. Hughes OTO, Gordon KC, Swann JC, et al: Pneumatosis cystoides intestinalis. Gut 7:553-557, Oct 1966 3. Keyting WS, McCarver RR, Kovarik JL, et al: Pneumatosis intestinalis: a new concept. Ra~iology 76:733-741, May 1961 4. Koss LG: Abdominal gas cysts (pneumatosis cystoides intestinorum hominis). An analysis with a report of a case and a critical review of the literature. Arch PathoI53:523-549, Jun 1952 5. Marshak RH, Blum SO, Eliasoph J: Pneumatosis involving left side of colon. JAMA 161:1626-1628, 25 Aug 1956 6. Meihoff WE, Hirschfield JS, Kern J Jr: Small intestinal scleroderma with malabsorption and pneumatosis cystoides intestinalis. Report of three cases. JAMA 204:854-858,3 Jun 1968 7. Mueller CF, Morehead R, Alter AJ, et al: Pneumatosis intestinalis in collagen disorders. Am J Roentgenol 115:300-305, Jun 1972 8. Seaman WB, Fleming RJ, Baker OH: Pneumatosis intestinaUs of small bowel. Semin RoentgenoI1:234-242, Apr 1966 9. Shapeero LG, Myers A, Oberkircher PE, et al: Acute reversible lupus vasculitis of the gastrointestinal tract. Radiology 112: 569-574, Sep 1974
Department of Radiology Hospital of the University of Pennsylvania 3400 Spruce Street Philadelphia, Pa. 19104
