Systemic rheumatic diseases are chronic, inflammatory autoimmune disorders, such as osteoarthritis, rheumatoid arthritis, systemic lupus erythematosus, and systemic sclerosis (scleroderma). ED patients with systemic rheumatic diseases have complex clinical and pharmacologic histories and multi–organ system pathology. Many extra-articular manifestations of rheumatic diseases can result in serious morbidity or mortality if not recognized and properly managed.1,2 This chapter discusses rheumatologic emergencies from an organ system perspective. It will review both rheumatologic emergencies as well as care for the rheumatologic patient in the ED. Table 282-1 categorizes emergencies associated with systemic rheumatic diseases. Although the chapter should be reviewed in its entirety, high-yield clinical pearls can be found at the end of the chapter in Table 282-8.
TABLE 282-1Categories of Emergencies in Patients With Systemic Rheumatic Diseases ||Download (.pdf) TABLE 282-1 Categories of Emergencies in Patients With Systemic Rheumatic Diseases
|Category ||Clinical Manifestations |
|Disease exacerbation ||Flare-up of preexisting systemic rheumatic disease |
|Complication ||Known complication of an acute systemic rheumatic disease (i.e., pulmonary hemorrhage, pericarditis, respiratory, or renal impairment) |
|Infection ||Infection complicated by immunosuppressive therapy |
|Comorbidity exacerbation ||Worsening or onset of a serious illness that is not a direct manifestation of the systemic rheumatic disease |
|Adverse drug reaction ||Reaction to a systemic rheumatic disease drug treatment |
CLINICAL FEATURES AND DIAGNOSIS
Table 282-2 lists typical clinical manifestations and complications specific to rheumatic diseases. The clinical descriptors may allow suspicion for a systemic rheumatic disease in a previously undiagnosed patient; however, the diagnosis cannot be confirmed during an ED visit. The diagnostic criteria and testing sequence, which are usually completed in the outpatient setting, are beyond the scope of this chapter. The need to admit a patient with a known or suspected systemic rheumatic illness depends on the severity of the patient’s presentation. In systemic rheumatic diseases, infection is the leading cause for intensive care unit admission followed by rheumatic disease flare.3 The intensive care unit admission may be the site of initial diagnosis in 20% of those with systemic rheumatic disease. Rheumatoid arthritis is the most common rheumatic disease seen in intensive care unit patients (also the most common rheumatic disease), followed in decreasing frequency by systemic lupus erythematosus and systemic sclerosis.
TABLE 282-2Common Features and Complications of Systemic Rheumatic Diseases ||Download (.pdf) TABLE 282-2 Common Features and Complications of Systemic Rheumatic Diseases
|Disorder ||Common and Characteristic Clinical Features ||Complications |
|Antiphospholipid syndrome ||Multiple and recurrent venous and arterial thromboses, recurrent early abortions. Secondary form is associated with systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis, and Sjögren’s syndrome. Thrombophlebitis and deep vein thrombosis, thrombocytopenia, hemolytic anemia, microangiopathic hemolytic anemia, livedo reticularis, stroke, transient ischemic attack, eye vascular complications. Coronary, renal, mesenteric, and cerebral vascular occlusion. ||ARDS, pulmonary embolism, ischemic complications of vascular occlusion, severe anemia, vision loss, catastrophic antiphospholipid syndrome. |