Abstract
Introduction. Pneumatosis intestinalis (PI) is an uncommon pathology characterised by the presence of gas within the intestinal wall. It has been associated with various conditions, including connective tissue diseases. This is the first report of PI being the initial presentation of systemic sclerosis. Case Presentation. The patient, a 75-year-old female, presented with an 8-month history of worsening dysphagia and epigastric pain, as well as other nonspecific symptoms. Initial investigations with an oesophagogastroduodenoscopy diagnosed Candida oesophagitis and also identified an extrinsic compression of the gastric antrum. Subsequently a CT scan of the abdomen and pelvis showed moderately dilated small bowel loops and PI. Due to the patient's stability, non-critical clinical condition, conservative management was instituted. More detailed investigations confirmed the diagnosis of systemic sclerosis with positive anticentromeric and antinuclear antibodies. The patient improved on methotrexate and was discharged with appropriate outpatient follow-up. Discussion. PI is a rare but well-documented pathology associated with connective tissue diseases, such as systemic sclerosis. In most cases, conservative management is preferable to surgical intervention, depending on the patient's clinical presentation and progress. This is the first report of PI being the initial presentation of a patient with systemic sclerosis responsive to conservative management.
Highlights
Pneumatosis intestinalis (PI) is an uncommon pathology characterised by the presence of gas within the intestinal wall, associated with a variety of clinical disorders [2, 4, 5], including connective tissue diseases [6]
PI has been described in progressive systemic sclerosis, reported as a late complication and a poor prognostic indicator [8]; systemic lupus erythematosus, where it is suggested to reflect ischemic necrosis of the bowel due to vasculitis [9]; mixed connective tissue disease [2, 7, 9,10,11], where it may be associated with a better prognosis [12, 13], while it is often associated with corticosteroid administration [14]
The bacterial theory supports that gas-producing bacteria, such as Clostridium difficile or Clostridium perfringens, invade the submucosal layer through mucosal rents and produce gas within the intestinal wall [16]
Summary
Pneumatosis intestinalis (PI) is an uncommon pathology, first described in 1730 [1], characterised by the presence of gas within the intestinal wall, usually in the mucosa and submucosal layers of the antimesenteric border [2]. Primary PI is a benign idiopathic condition [3], while secondary PI can be associated with various underlying conditions, including necrotising enterocolitis, obstructive lung disease, inflammatory bowel disease, and connective tissue diseases [4,5,6]. Even though the association between PI and connective tissue diseases is well known [2, 7], antiphospholipid syndrome is the only mixed connective tissue disorder in which, it has been reported as the initial presentation [8]. To our knowledge, this is the first report of PI being the initial presentation of systemic sclerosis
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