Lupus-associated vasculitis manifesting as acute appendicitis in a 16 year old girl
© Cellini et al; licensee BioMed Central Ltd. 2008
Received: 16 July 2007
Accepted: 27 June 2008
Published: 27 June 2008
A 16 year old female with systemic lupus erythematosus presents with acute appendicitis. Final pathologic analysis of the appendix describes a lupus-associated vasculitis.
Acute abdomen in patients with systemic lupus erythematosus (SLE) can pose a diagnostic and therapeutic challenge. Most of these patients are on chronic steroid treatment which may mask symptoms and lead to delay in diagnosis. Delay in diagnosis and treatment can increase mortality. Lupus-associated vasculitis is reported to be the cause of acute abdomen in up to 60% of SLE patients . We report a case of a female patient who was referred to us with symptoms of acute abdomen due to appendicitis associated with vasculitis.
Patients with SLE presenting with an acute abdomen pose a unique diagnostic dilemma for surgeons, as the differential diagnosis is diverse and often difficult to make. Usually these patients are on chronic steroid treatment making physical exam and laboratory values unreliable therefore leading to a delay in diagnosis and treatment. Surgical intervention is fraught with many potential complications especially in patients with severe disease activity who require chronic steroid and immunosuppressive therapies. Analysis of lupus patients with an acute surgical abdomen failed to identify any clinical, laboratory or radiological features that could reliably aid in early diagnosis and management . In addition it can be difficult to assess whether the cause of the pain is secondary to lupus peritonitis or to an acute surgical process .
Studies have found that vasculitis is the cause of acute abdomen in 35–60% of cases of SLE [1, 4–6]. Causes of vasculitis- associated acute abdomen in SLE patients include intestinal ischemia and necrosis, pancreatitis and cholecystitis. Acute appendicitis is usually considered a non-SLE-related cause of acute abdomen and is considered separate from the vasculitits- associated cases. Our case, however, suggests that there might be a cause and effect relationship between the two. Review of the literature reveals two European case reports of two female patients in their twenties with SLE presenting with abdominal complaints which ultimately were the result of appendicitis following autoimmune vasculitis [7, 8]. Few other reports exist linking autoimmune associated vasculitis with appendicitis, cholecystitis, bowel perforation or ischemia [1, 9–12].
The typical proposed pathogenesis of appendicitis is obstruction causing increased intraluminal pressure and subsequent collapse of the draining vessels. The ischemic injury that follows favors bacterial proliferation and additional inflammatory exudate and edema leading to the typical clinical manifestations of appendicitis. It is theorized that the lupus-associated vasculitis can follow a similar pathogenetic pathway by causing ischemia, bacterial proliferation and inflammation .
Regardless of pathology, delay in diagnosis can be dangerous and a high index of suspicion must always be maintained when dealing with such patients. Early laparotomy shows an increase in survival rate . Emergent diagnostic laparoscopy has been advocated by some as a useful way to diagnose and allow for treatment of acute abdomen in SLE .
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