Page 1776 - Hall et al (2015) Principles of Critical Care-McGraw-Hill

CHAPTER 126: Rheumatology in the ICU 1245

dramatic subluxations on MRI are not accompanied by neurologic approximately 10% of cases of FUO. Because rheumatoid arthritis,
signs or symptoms. Flexion and extension films of the cervical spine scleroderma variants, dermatomyositis, polymyositis, and polymyalgia
may show dynamic instability and subluxation of C1 on C2. There are rheumatica are not usual causes of significant fever, they need not be
few data about the specificity or sensitivity of such films to predict a strongly considered. SLE can present with high fevers, either spiking
cervical cord catastrophe. Clearly, caution should be exercised in the or relatively constant, and leukopenia, hypoalbuminemia, anemia, and
intubation of patients with rheumatoid arthritis and neck disease; if time an elevated erythrocyte sedimentation rate (ESR), but few other overt
allows, nasotracheal or fiberoptically guided endotracheal intubation is clinical signs of lupus such as rash or serositis, polyarthritis, or active
preferred. Cervical instability is a problem that attends advanced urinary sediment. Most systemic necrotizing vasculitides will be evident
destructive rheumatoid arthritis. Early aggressive treatment of RA after examination of the skin, chest radiograph, and urinary sediment.
patients with disease-modifying therapy has dramatically reduced the An extremely high ESR is a nonspecific laboratory clue in FUO, but anti-
prevalence of this problem. 25 bodies to relevant specific antinuclear or streptococcal disorders provide
supportive evidence. A most vexing diagnosis to pin down is that of adult
AUTOINFLAMMATORY DISEASES Still’s disease. These patients will have relentless spiking fevers, at times
a history of FUO in childhood, leukocytosis, elevated levels of ferritin,
Autoinflammatory diseases are rare conditions that have episodic mild to moderate hepatic enzyme changes, and an occasional truncal
features of inflammation with little or no evidence of systemic auto- rash. Polyarthritis or arthralgias are not a constant feature early in this
antibodies or autoreactive T lymphocytes. 26,27 These diseases com- syndrome. Hepatosplenomegaly and lymphadenopathy are common.
monly have episodic fever that can suggest infection or malignancy Rarely, acute pericarditis with tamponade or myocarditis with respira-
and include diseases such as adult-onset Still’s disease, systemic-onset tory failure may complicate the course of adult Still’s disease. In the
30
juvenile idiopathic arthritis, familial Mediterranean fever, and perhaps final analysis, treatment may have to be based on the supposition that
Behcet syndrome. Whereas many of the systemic autoimmune diseases the patient has relentless, immunologically driven, noninfectious inflam-
we recognize (eg, rheumatoid arthritis, systemic lupus erythematosus) mation that evades specific diagnosis. Blanket suppression of cytokines
come about due to abnormalities affecting the adaptive immune system, and white blood cell responses by corticosteroids may be necessary. The
the autoinflammatory diseases appear to arise because of abnormali- steroid dose is tailored to control fever and normalize the acute-phase
ties in the innate immune system. Interleukin-1 helps “supervise” early response. Anakinra and Tocilizumab have also been used for therapy.
immune responses and appears to be central to the development of ■
clinical disease in autoinflammatory syndromes. Recognition of the ISCHEMIC HEART DISEASE IN THE RHEUMATOLOGY PATIENT
importance of IL-1 has focused attention on therapeutic value of inhib- Premature atherosclerosis can be seen in association with systemic auto-
iting IL-1. Anakinra, the IL-1 receptor antagonist, has gained attention immune disease; RA and SLE have been documented well. Traditional
31
in treating conditions such as familial Mediterranean fever and perhaps (Framingham) risk factors for cardiovascular disease only partially
adult-onset Still’s disease in addition to potentially being another thera- account for this accelerated atherosclerosis. Myocardial infarction
32
peutic option in Behcet disease. makes a major contribution to excessive mortality in SLE. Mortality data
suggest a bimodal distribution with many late deaths in SLE secondary
OUTCOMES OF PATIENTS WITH RHEUMATOLOGIC to ischemic heart disease. The primary cause of ischemic heart disease
DISEASES IN THE ICU in lupus is atherosclerosis. The cause of accelerated atheroma is unclear,
but inflammation of the vascular endothelium due to chronic immune
Mortality rates for patients with systemic rheumatic diseases admitted complex disease compounded by the effects of corticosteroids is likely
to the ICU are high. This is perhaps not unexpected when consid- to be a major contributing factor. Vasculitis in SLE may affect any organ
1
eration is given to a typical scenario of a chronically ill patient with including the heart although the frequency is not high. Identifying myo-
multiple impaired organs, a disordered immune system, treatment with cardial ischemia caused by vasculitis (as opposed to premature athero-
chronic immunosuppression, and admission to the ICU with infection. sclerosis) can be difficult; however, most patients with coronary artery
Reported ICU mortality rates for patients with rheumatic disease are vasculitis have evidence of active vasculitis in other organs. Serologic
higher than would be predicted using the Acute Physiology, Age, and evidence of active lupus and markers of systemic inflammation includ-
Chronic Health Evaluation II (APACHE II) Score or Simplified Acute ing low hemoglobin and albumin are likely to be present. Acute therapy
Physiology Score II (SAPS II). 28,29 Factors associated with poor outcome would include high-dose corticosteroids. In the face of widespread
include higher SAPS II scores, poor health status prior to admission, vasculitis, additional intervention with parenteral immunomodulatory
duration of rheumatic disease, corticosteroids and immunosuppressive therapy may be indicated. 33
drugs, renal failure, coma, and acute respiratory distress syndrome. The
overall ICU mortality rate for patients with rheumatic disease ranges
from 30% to 60%. Mortality rates are higher for patients admitted MULTIPLE AUTOANTIBODIES AND MULTISYSTEM
1
because of infection compared to those admitted for exacerbation of INFLAMMATORY DISEASE: WHAT NAME DO I GIVE IT?
rheumatic disease. 28,29 Since infection may cause two-thirds of ICU The systemic autoimmune diseases are characterized by the presence
admissions among patients with rheumatic disease, aggressive intense of sterile inflammation in multiple organs and multiple autoantibodies.
therapy of the febrile patient with broad-spectrum antibiotic coverage The prototypical disease is SLE, which is characterized by the widest
is appropriate. clinical and serologic spectrum. Other diseases include scleroderma,
■ FEVER OF UNDETERMINED ORIGIN: RHEUMATIC CAUSES Sjögren syndrome, polymyositis, dermatomyositis, rheumatoid arthri-
tis, and syndromes with overlapping features (overlap syndrome or
The traditional definition of a fever of undetermined origin (FUO) undifferentiated connective tissue disease). The clustering of clinical fea-
depicts a patient with a significant fever of 6 weeks or greater duration tures and the nature and diversity of autoantibodies may strongly suggest
and no definable cause. In actual practice, depending on the impatience one disorder rather than another, but the overlap of clinical, serologic,
of the attending physician, FUO usually becomes the working diagnosis and pathologic features among these diseases can be large, leading to
within 3 to 14 days after a fruitless search for classic causes of pyrexia. considerable consternation for clinicians. Debates about whether a given
The most common causes of FUO are occult infection, drugs, and patient has SLE, primary Sjögren syndrome, or an overlap syndrome are
occasionally, malignancy. After those are excluded, one must consider tiring, usually unresolvable, and generally irrelevant. The therapy for the
a limited array of rheumatic diseases that could be present in a febrile immunologically active phase of these disorders is not disease specific.
patient with protracted fever. Rheumatic disorders may account for The absence of a consensus label should not delay therapeutic efforts.

section11.indd 1245 1/19/2015 10:52:15 AM