Page 1777 - Hall et al (2015) Principles of Critical Care-McGraw-Hill

1246 PART 11: Special Problems in Critical Care

THE ELDERLY PATIENT WITH AN ELEVATED urgent laparotomy with resection of affected areas. Medical therapy
37
SEDIMENTATION RATE: IS THIS GIANT CELL ARTERITIS includes supportive therapy and intravenous corticosteroids; cyclophos-
phamide is often added. In patients with vascular infarction, known to
(TEMPORAL ARTERITIS)?
have antiphospholipid antibodies, anticoagulation may be indicated.
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Within the ICU, advanced age, elevation of the ESR, anemia, and various Rituximab, IVIg, and TNF antagonists have been used in refractory
nonspecific clinical features—chiefly fever—may converge to raise the cases and the role of plasma exchange or plasmapheresis is unclear. 35,37
question of temporal arteritis. Temporal arteritis is a granulomatous vas-
culitis that affects those over age 60 (mean age 70) and has a proclivity LUNG INFILTRATES IN RENAL FAILURE: IS THIS AN
to affect extracranial vessels and branches of the aorta. Myriad uncom-
mon clinical features may develop, but headache, polymyalgia rheu- IMMUNE-MEDIATED PULMONARY-RENAL SYNDROME?
matica, visual disturbance, scalp tenderness, and jaw claudication are the When a patient has abnormalities of both renal and pulmonary systems,
common fingerprints of this disease. Treatment is highly effective with an immune-mediated pulmonary-renal syndrome should be strongly
prednisone in doses of 40 to 60 mg/d. The onset of this disease is typi- considered and ruled out as soon as possible. Examples of pulmonary-
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cally insidious, and its complications rarely prompt admission to the ICU. renal syndromes include SLE, GPA, microscopic polyangiitis, advanced
Therefore, the question is more commonly framed in terms of whether cardiac failure, infection, and Goodpasture syndrome. Goodpasture
the patient has developed new-onset temporal arteritis in the ICU (prob- syndrome is characterized by antibodies to both alveolar and glomeru-
ably not) or did they have it as a comorbid state at admission? History lar basement membranes (GBM). The diagnosis rests on establishing
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and old records are critical in this regard. Temporal artery biopsy is the the presence of anti–basement membrane antibodies in the peripheral
gold standard, although sensitivity varies with the institution. A classical blood or in situ deposition in a renal biopsy. Characteristic histologic
clinical scenario that includes headache, visual symptoms, jaw claudica- findings in the renal biopsy are those of a diffuse proliferative necrotiz-
tion, scalp tenderness, and polymyalgia rheumatica may be sufficiently ing glomerulonephritis highlighted by a somewhat unique characteristic
compelling to prompt treatment. Usually the situation is murkier. There of rather exuberant crescent formation. Although not well documented
is no substitute for the temporal artery biopsy, which can be done under by contemporary clinical studies, immunohistologic analysis of tissue
local anesthesia with little morbidity. A sufficiently large piece (3-4 cm) obtained by the transbronchial route has not been helpful in most
should be obtained and adequate cuts done for pathology. Contralateral instances. The diagnosis of an antibody-mediated pulmonary-renal
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biopsy is done routinely by some if a first biopsy is negative. That deci- syndrome is important because efficacious therapy exists, especially when
sion will be influenced by the details of the clinical scenario and the risk initiated early in the disease course. Plasmapheresis, corticosteroids, and
of an empiric trial with steroids. A negative biopsy in a marginal clinical cyclophosphamide have formed the cornerstone of therapy. Small
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situation is reasonable grounds to withhold therapy. studies have shown efficacy with rituximab, and recalcitrant disease
40
has been treated with mycophenolate and cyclosporine. Anti-neutrophil
ABDOMINAL PAIN AND ELEVATED ERYTHROCYTE cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) associ-
SEDIMENTATION RATE: IS THIS VASCULITIS? ated respiratory disease consists of GPA, microscopic polyangiitis, and
Churg-Strauss vasculitis. GPA is associated with antibodies to proteinase
The clinical presentation of abdominal pain in association with an 3 (PR3). Microscopic polyangiitis and Churg-Strauss vasculitis can
elevated ESR raises the diagnostic possibility of vasculitis. If the patient be associated with anti-myeloperoxidase antibodies (MPO). Alveolar
is already known to have systemic vasculitis, the differential diagnosis of hemorrhage is the main cause of hospitalization and ICU admission.
the abdominal pain and elevated ESR is a bit more straightforward. In Clinically, GPA can cause hoarseness, cough, dyspnea, stridor, wheezing,
the unusual situation when a patient with relatively limited or no vascu- hemoptysis, cavitary lesions, alveolar hemorrhage, bronchiectasis, lung
litic findings in skin, renal, neurologic or pulmonary systems develops nodules, or infiltrates. Churg-Strauss vasculitis may cause nasal obstruc-
fulminant symptoms affecting the bowel, the diagnosis may remain tion, recurrent sinusitis, or nasal polyposis. In addition, 95% of patients
elusive until angiography or surgical exploration is performed. Patients with Churg-Strauss vasculitis will have (or have had) asthma. Treatment
already on moderate to high dose corticosteroid therapy for pre-existing is with corticosteroids and cyclophosphamide. Rituximab has recently
systemic vasculitis may develop bowel ischemia or perforation with been approved for the treatment of GPA and plasma exchange has been
relatively few physical findings. Mesenteric vasculitis can occur in advocated in particularly aggressive disease. 41
polyarteritis nodosa, microscopic polyangiitis, mixed cryoglobuline- Up to one-third of patients with ANCA-associated disease (typically
42
mia and occasionally in granulomatous polyangiitis (GPA—formerly a p-ANCA) will have GBM antibodies. The estimated incidence of the
referred to as Wegener disease), Churg-Strauss and Henoch-Schonlein combination of Anti-GBM and ANCA is 0.47 per million people and the
purpura. Diarrhea and profound protein-losing enteropathy and acute prognosis for those with dual antibodies is similar to those with GBM-
bowel obstruction secondary to adhesive serositis have occurred rarely antibody disease. 38
in SLE. Acute pancreatitis has also been observed in the patient with
SLE; appropriate biochemical and imaging assessment will confirm this CNS DYSFUNCTION: IS THIS VASCULITIS?
diagnosis. Abdominal pain is the most common presenting symptom in
those ultimately found to have mesenteric vasculitis and approximately Patients with or without connective tissue disease and nonspecific mark-
30% will present with an acute (or surgical) abdomen. Gastrointestinal ers of systemic inflammation who develop signs of brain dysfunction
hemorrhage is uncommon, occurring in less than 30%. Gastrointestinal are routinely suspected of having CNS vasculitis. Often the likelihood is
tract involvement in the setting of systemic vasculitis has a particularly considered low but addressed in the interest of being thorough. Rarely
bad outcome; however, mortality rates appear to have decreased since does CNS vasculitis cause psychosis or coma without focal neurologic
the 1970s. The evaluation of these patients traditionally started with signs. Primary angiitis of the CNS, particularly in early stages, is an
35
plain abdominal films which might show free air or distended bowel exception, but would not be expected to show signs or symptoms
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loops. The relatively low sensitivity for finding these lesions has led to of multisystem disease or coma that would prompt ICU admission.
a more common use of computed tomography (CT) early in the evalu- Finding focal deficits on examination is difficult in the comatose or
ation. Endoscopic evaluation (with biopsy) may have an appearance disoriented patient. A skilled neurologist can contribute more than
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suggestive of ischemia but biopsies have a low sensitivity to diagnose imaging techniques. MRI and CT scanning may reveal a lesion sug-
vasculitis. Angiography may be necessary to make an appropriate gesting an ischemic event. While no pattern is specific for vasculitis, a
35
diagnosis in less urgent situations. The role of MRI and/or MR angiog- completely normal MRI coupled with normal cerebrospinal fluid makes
raphy is still unclear. Patients with mesenteric ischemia should undergo the diagnosis less likely. Angiography can be very helpful in identifying

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