Page 722 - Clinical Immunology_ Principles and Practice ( PDFDrive )

CHaPtEr 51 Systemic Lupus Erythematosus 695

frequently associated with disease activity. Histologically, there vasculitis preferentially affects the superior mesenteric artery,
is a peribulbar lymphocytic infiltrate surrounding shrunken involving the small intestine more commonly than the large
anagen hair bulbs similar to findings in alopecia areata. Nonscar- bowel. Vasculitis can also occur in the esophagus, stomach,
ring alopecia resolves with complete hair regrowth with control peritoneum, rectum, gallbladder, pancreas, and liver. Computed
of disease activity. tomography (CT) and/or magnetic resonance with or without
A wide spectrum of nail abnormalities, including pitting, angiography are the preferred imaging tests for evaluation of
ridging, onycholysis, and dyschromia with blue or black hyper- abdominal pathology; the radiographic signs of intestinal ischemia
pigmentation, are reported in up to 30% of SLE patients, but do not differ based on pathogenesis.
none are lupus-specific. Nail fold erythema with ragged cuticles In cases with an insidious clinical course, endoscopy and
and splinter hemorrhages resembling the changes of dermato- colonoscopy may provide evidence of ischemia demonstrating
myositis are common. ulcerating or heaped-up lesions with overt vasculitis on biopsy.
The lesions are segmental and focal. Histologically, there is a
Oral Lesions small-vessel arteritis and venulitis with neutrophilic, lymphocytic,
The spectrum of oral lesions reported in SLE includes cheilitis, and macrophage infiltrates and fibrinoid necrosis of the vessel
ulcerations, erythematous patches, lichen planus–type plaques walls, associated thrombosis, and mononuclear infiltrate in the
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on the buccal mucosa and palate, and DLE. Most oral lesions lamina propria. There may be immunoglobulin, C3, and fibrin
are asymptomatic and must be looked for on examination. Positive deposition in the adventitia and media.
immunofluorescent staining on biopsy may be useful to differenti- Timely treatment with high-dose intravenous corticosteroids
ate DLE from lichen planus–like lesions and leukoplakia. Lupus (1–2 mg/kg per day) usually results in a favorable outcome and
mucosal ulcerations demonstrate an interface mucositis and not avoidance of serious complications such as bowel necrosis and
leukocytoclastic vasculitis. perforation. To date there are no randomized trials of immu-
nosuppressive agents for the treatment of intestinal vasculitis.
Gastrointestinal Manifestations
GI symptoms occur commonly in SLE with reported incidences Intestinal Pseudoobstruction
of 15–75%; attribution is critical, as at least half are Though rare, SLE-associated intestinal pseudoobstruction
attributable to side effects of medications and to infectious (SLE-IPO) may be the initial manifestation of SLE. 51,53 Clinical
complications. 51 symptoms (abdominal pain and distension with diminished or
absent peristalsis) and radiographic findings of SLE-IPO mimic
Esophagus those of mechanical obstruction. Distinguishing features of
The prevalence of esophageal involvement varies. Many reviews SLE-IPO include concomitant active SLE in other organ systems
citing a high incidence of dysphagia and odynophagia predate and associations with hematological cytopenias, hypocomple-
the advent of proton pump inhibitors and H 2 blockers, and the mentemia, and serositis. Findings of coexisting ureterohydro-
relationship of medication use to symptoms is not clear. Dysphagia nephrosis and hepatobiliary dilatation in the absence of
and heartburn are reported in 1–50% of patients, although obstructing lesions suggest underlying smooth muscle dysmotility.
esophageal dysmotility is observed in up to 72%. An inflammatory Poor prognosis is associated with older age at SLE diagnosis, GI
process involving esophageal muscle or vasculitic damage to the symptoms as the initial SLE manifestation, longer disease duration,
Auerbach plexus is thought to contribute to the esophageal and delayed diagnosis of IPO.
dysmotility. Ulceration is rarely seen outside the context of
infections such as invasive candidiasis, herpes simplex, or Peritonitis
cytomegalovirus. SLE patients with a secondary Sjögren syndrome Inflammation of serosal membranes is well described in SLE;
may have salivary gland dysfunction, resulting in decreased saliva despite evidence of peritoneal inflammation in 63% of autopsy
contributing to dysphagia. studies, symptomatic pericarditis and pleuritis occur far more
commonly than peritonitis. Acute peritonitis may be attributed
Abdominal Pain/Vasculitis to peritoneal vasculitis or ischemia and presents with abdominal
Acute abdominal pain is common in SLE with reported incidences pain (see above). The finding of ascitic fluid by CT scan or
as high as 40%. The differential diagnosis includes intestinal ultrasound mandates an evaluation of the fluid to exclude infec-
vasculitis (45.5%), pancreatitis (10.8%), hepatobiliary disease tion and malignancy. Rarely, ascites may be attributable to hepatic
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(18.8%), and intestinal pseudoobstruction (IPO) (3.3%). The or portal vein thrombosis. Chronic peritonitis characterized by
most catastrophic and potentially fatal GI disturbances are related large amounts of painless ascites attributable to SLE and not to
to ischemia of the small and large intestines resulting from heart failure, constrictive pericarditis, or severe hypoalbuminemia
medium- and small-vessel vasculitis or thrombotic complications due to nephrotic syndrome, liver disease, or a protein-losing
of antiphospholipid (APL) antibodies. Approximately half of enteropathy (PLE) is rare. In lupus peritonitis, the ascitic fluid
SLE patients with acute abdominal pain will have intestinal is generally exudative with a predominance of lymphocytes; LE
ischemia; associated mortality is high, so early consideration cells, autoantibodies, and low complement levels are frequent.
and intervention is critical. Intestinal vasculitis is frequently On biopsy, the peritoneum is usually edematous; it is sometimes
associated with active disease elsewhere and increased SLE hemorrhagic with lymphocytic perivascular infiltrates.
Disease Activity Index (SLEDAI) scores, whereas SLE patients
with inactive disease and acute abdominal pain will have different Pancreatitis
intraabdominal pathology unrelated to SLE. The clinical presenta- Pancreatitis attributable to SLE is rare, occurring in 8–11% of
tion may be acute, severe abdominal pain or an insidious, patients with abdominal pain and having an annual reported
stuttering course with nausea, vomiting, bloating, diarrhea, incidence ≤1/1000. Although both corticosteroids and azathioprine
postprandial fullness, anorexia, and weight loss. Mesenteric can trigger pancreatitis, 34% of reported cases are not on these