Pneumatosis Cystoides Intestinalis with Systemic Sclerosis, Limited Type Resulting in a Poor Prognosis
ABSTRACT Pneumatosis cystoides intestinalis (PCI) is a rare disease characterized by the presence of multilocular intramural clusters of gas in the alimentary tract and has been considered to have a favorable response to conservative treatment. We describe the first case of limited type of systemic sclerosis (SSc) with PCI. A 74-year-old Japanese woman presented with a 4-month history of an unhealed cutaneous ulcer on the right third finger, along with sclerodactyly of bilateral hands. Proximal skin sclerosis was absent. The patient reported acute abdominal pain, and a diagnosis of PCI was established on plain radiography. The patient died of multiple organ failure 5 months after the development of PCI. PCI is rarely complicated with SSc, and all cases previously reported were associated with diffuse SSc. Because PCI is one of the poor prognostic factors of SSc, we should recognize the presence of this condition even in patients with limited cutaneous involvement.
- The Journal of Dermatology 02/2008; 35(1):49-51. DOI:10.1111/j.1346-8138.2007.00414.x · 2.35 Impact Factor
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ABSTRACT: Systemic sclerosis (SSc, scleroderma) is a chronic, connective tissue disease characterized by vascular damage and fibrosis of the skin and internal organs including the gastrointestinal (GI) tract. After skin involvement, the GI tract is the second most commonly involved organ system with the esophagus being the most frequent segment involved. Involvement of the stomach, small intestine, colon, and anorectum is less common but may lead to severe complications and debility. Ultimately, over 80% of individuals with scleroderma will experience a change in normal GI function (Szamosi S, Szekanecz Z, Szucs G. Gastrointestinal manifestations in Hungarian scleroderma patients. Rheumatol Int. 2006;26:1120–4; Weston S, Thumshirn M, Wiste J, et al. Clinical and upper gastrointestinal motility features in systemic sclerosis and related disorders. Am J Gastroenterol. 1998;93:1085–9).Scleroderma, 01/2012: pages 463-469; , ISBN: 978-1-4419-5773-3
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ABSTRACT: Pneumatosis cystoides intestinalis (PCI) is a rare life-threatening gastrointestinal complication in the course of connective tissue disease (CTD). PCI is characterised by the appearance of intramural clusters of gas in the small and large bowel wall on X-ray or computed tomography and often is accompanied by free air in the peritoneal cavity. We present three cases of PCI in patients with scleroderma-related conditions. A review of the English language literature published on MEDLINE from 1973 to 2008 was conducted using the terms: 'systemic sclerosis', 'connective tissue disease' and 'pneumatosis cystoides intestinalis'. This review focused on clinical features, diagnostic and treatment strategies of PCI in the context of CTD. Symptoms of PCI are non-specific: abdominal pain, vomiting, constipation, bloating and weight loss. Coexistence of PCI with other manifestations of CTD, such as intestinal pseudo-obstruction and/or bacterial overgrowth, complicates the clinical diagnosis. Treatment approach to PCI is mostly conservative: intestinal 'rest', parenteral nutrition, antibiotics, fluids and electrolyte supplementation, and inhaled oxygen. Surgical intervention should be performed only in cases of bowel perforation, ischaemia or necrosis. Patients with PCI have high mortality rates due to PCI itself but also to the severity and variety of basic CTD complications. Recognition of PCI, particularly in the context of underlying CTD, is necessary for proper therapeutic application. In patients with underlying CTD and symptoms of abdominal emergency, recruitment of multidisciplinary teams, including rheumatologist, gastroenterologist, imaging specialist and surgeons familiar with intestinal complications of CTD-related conditions, is warranted.Internal Medicine Journal 03/2012; 42(3):323-9. DOI:10.1111/j.1445-5994.2011.02557.x · 1.70 Impact Factor