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CHAPTER 126: Rheumatology in the ICU 1243

vasculitis presents a diagnostic and therapeutic dilemma for the criti- tended to be reversible as reported in the literature but occasionally can
cal care clinician. Involvement of the central nervous system in SLE lead to permanent deficits or infarction. 11
(NeuroPsychiatric SLE or NPSLE) occurs in approximately 30% to A difficult but critical differentiation must be made in SLE patients
40% of patients with SLE. Common presentations of NPSLE include with CNS findings, anemia, and thrombocytopenia. The latter find-
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headache, cognitive dysfunction, mood disorders, seizure disorders, and ings, while very common in active SLE, coupled with peripheral blood
cerebrovascular disease (strokes and transient ischemic attacks are the smear evidence of microangiopathy, point instead to thrombotic throm-
most common). A common dilemma is to differentiate between steroid- bocytopenic purpura (TTP). TTP is uncommon with an estimated
induced mental status/mood changes and those owing to active SLE. incidence of 4 to 11 cases per million people. The diagnosis requires
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Risk factors for the development of NPSLE include other SLE activity or microangiopathic hemolytic anemia (MAHA) and thrombocytopenia.
damage, presence of antiphospholipid antibodies and previous or con- Only 50% patients will have neurological symptoms such as seizures
current NPSLE, increasing age, hypertension, hyperlipidemia and other or focal deficits. Although part of the classic pentad, fever is uncom-
psychiatric distress. The evaluation of a patient with suspected NPSLE mon and should stimulate a search for infection. Increased LDH,
starts with a complete history and physical examination with attention indirect bilirubin, and negative Coomb test are consistent with MAHA.
to excluding non-SLE-related conditions. Depending on the patient’s ADAMTS13 levels have been associated with the pathophysiology of
symptoms, further evaluation can include complete blood counts, bio- many cases of TTP; however, results may not be immediately available.
chemical and serologic tests, examination of the CSF, and MRI of the Identifying TTP in the ICU setting is further complicated by the fact that
brain and/or spinal cord. Measurement of serum antiphospholipid anti- critical care patients are usually anemic and thrombocytopenic for other
bodies can be clinically very useful, particularly in a patient with focal reasons (such as severe sepsis). Ten percent of patients with TTP may have
neurologic symptoms or signs. CSF abnormalities (pleocytosis, protein concomitant cancer or sepsis. Once TTP is diagnosed, patients should
elevation and low glucose levels) support the diagnosis of NPSLE but undergo plasma exchange until platelets are normalized. Case reports
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are not specific. CSF levels of IL-6, oligoclonal bands, and IgG indices suggest that TTP and SLE can co-occur, and the differentiation between
can also be useful in identifying immunologic activity involving the the two diseases is vital since the treatment of life-threatening TTP is
CNS. In general, therapy of active NPSLE starts with addressing general, plasma exchange and not concomitant pulse methylprednisone, alkylat-
aggravating factors (eg, hypertension, adverse drug effects, infectious or ing agents, and/or plasmapheresis that many rheumatologists, in spite
metabolic complications). Symptomatic therapy targeted to the patient’s of unproven benefit, may resort to in the setting of fulminant CNS SLE.
CNS problems are considered next, such as anticonvulsants in a seiz-
ing patient, antidepressants in depression, antipsychotic medications SCLERODERMA
in a patient with psychosis or antiplatelet/anticoagulation in patients
with NSPLE manifestations attributed to antiphospholipid antibodies. ■ PULMONARY HYPERTENSION
Immunomodulatory therapy has been used in aggressive cases of NPSLE
and includes corticosteroids, azathioprine, and/or cyclophosphamide. The emergence of effective therapeutic options has given the detection
Refractory or severe cases of NPSLE have prompted the use of of pulmonary vascular disease a new sense of urgency. The exact preva-
IVIg, plasma exchange, and rituximab which have been reported in lence of pulmonary hypertension in scleroderma is unknown but has
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uncontrolled studies. 8 been estimated to be 8% to 12%. There are generally two settings in
Four CNS disorders associated with SLE can be puzzling: (1) A which it is identified. In patients with limited cutaneous systemic scle-
small subset of patients with SLE who have taken NSAIDs, especially rosis (previously called CREST syndrome), it occurs classically as an iso-
ibuprofen, will develop a meningitis-like picture that is character- lated phenomenon in the absence of pulmonary fibrosis. This generally
ized by fever, severe headache, nuchal rigidity, and cerebrospinal fluid occurs in the second decade of disease or later. Patients who fall into the
pleocytosis. In an immunosuppressed patient, these findings prompt second major category of scleroderma with diffuse cutaneous involve-
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consideration of both common and unusual bacterial and fungal eti- ment may develop pulmonary hypertension as the result of advanced
ologies. The syndrome will remit rapidly once the drug is discontinued. pulmonary fibrosis. In both settings, the vascular disease is character-
(2) Headaches (including migraine) can be frequent in patients with ized by bland endothelial proliferation and vascular occlusion. The
SLE (up to 50% in some studies). High-risk features that would require vasculopathy of scleroderma is not characterized by an inflammatory
additional attention and evaluation include explosive onset, severe infiltrate and is not treated with corticosteroids or immunosuppression.
symptoms, age of onset over 50 years, fever, immunosuppression, pres- Early symptoms of pulmonary hypertension are exertional breathless-
ence of antiphospholipid antibodies, the use of anticoagulants, focal ness, but later symptoms could provoke admission to the ICU. These
neurologic findings, obtundation, meningismus, or other SLE activity. include near-syncope or syncope with exertion, angina, and right heart
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(3) Rare instances of myelopathy can occur in the context of active failure. Scleroderma patients, especially those at greatest risk for pul-
SLE, or even as the initial manifestation. The optimal therapy of this monary hypertension, are typically screened yearly or every few years
disorder is not clear but a retrospective review from 2000 by Kovacs using Doppler echocardiography. Confirmation of diagnosis demands a
et al suggested that aggressive, early therapy with intravenous methyl- right heart catheterization, which provides additional important infor-
prednisone followed by cyclophosphamide had the best outcomes. The mation about pulmonary capillary wedge pressure and cardiac output.
role of plasmapheresis was not clear. In those patients with antiphospho- The treatment of pulmonary hypertension in the context of scleroderma
lipid antibodies, a coagulopathic etiology has been postulated; however, it includes prostanoids (epoprostenol, iloprost, treprostinil), phosphodies-
is unclear whether the use of anticoagulation improves the outcome. terase-5 inhibitors (sildenafil, tadalafil), endothelin receptor antagonists
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(4) Posterior reversible, encephalopathy syndrome (PRES) is a syndrome (bosentan, ambrisentan), and general supportive therapy. End-stage
in which patients can present with headache, seizures, changes in vision pulmonary hypertension may require heart-lung transplantation. Other
and changes in mental status who have a characteristic MRI appearance. causes of pulmonary hypertension should not be overlooked in sclero-
Imaging shows hyperintensities on T2-weighted scans in the posterior derma patients, including recurrent thromboembolic disease.
PRES can be seen in acute hypertension, acute kidney injury, or in those ■ HYPERTENSIVE RENAL CRISIS
cerebral area. In addition to being seen in patients with active SLE,
taking immunomodulatory medications. Management of PRES can be Hypertensive renal crisis in patients with scleroderma has been a major
challenging. If a medication is implicated, discontinuation of the drug cause of mortality, so rapid recognition is crucial. Scleroderma renal
with management of the seizures and hypertension is indicated. If crisis (SRC) typically develops in patients with diffuse cutaneous disease
active SLE is implicated, intravenous methylprednisolone and cyclo- and rarely in patients with limited cutaneous disease. 14,15 Antibodies to
phosphamide have been advocated. The manifestations of PRES have RNA Polymerase III have also been associated with the development

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