[Pneumatosis cystoides intestinalis in systemic diseases: 3 cases]

Area of Science:

• Gastroenterology
• Rheumatology
• Internal Medicine

Background:

• Pneumatosis cystoides intestinalis (PCI) is a rare condition characterized by gas-filled cysts in the intestinal wall.
• While often linked to gastrointestinal or respiratory issues, PCI can also manifest as a complication of systemic diseases, most commonly systemic sclerosis.
• This abstract explores the association between PCI and various systemic autoimmune and inflammatory conditions.

Observation:

• Three patients with systemic vasculitis (temporal arteritis, polyarteritis nodosa) treated with prednisone developed PCI.
• One patient experienced a complication of retropneumoperitoneum.
• All patients achieved a favorable outcome with medical management.

Findings:

• A review of 62 reported cases reveals systemic sclerosis as the most frequent associated condition (45%).
• Other implicated systemic diseases include systemic lupus erythematosus, mixed connective tissue disease, dermatopolymyositis, polyarteritis nodosa, rheumatoid arthritis, Sjögren's syndrome, and amyloidosis.
• Corticosteroid therapy and digestive tract vasculitis are identified as primary causal factors in non-scleroderma cases.

Implications:

• PCI associated with systemic diseases, particularly those treated with corticosteroids, requires careful monitoring.
• Medical management, including corticosteroids, appears effective in treating PCI in these patient populations.
• Surgical intervention is rarely necessary, emphasizing the importance of conservative treatment strategies for this condition.