Chapter 282: Systemic Rheumatic Diseases

INTRODUCTION

Systemic rheumatic diseases are chronic, inflammatory, autoimmune disorders, such as rheumatoid arthritis, systemic sclerosis (scleroderma), or systemic lupus erythematosus. 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. This chapter discusses rheumatologic emergencies from an organ system perspective. Table 282-1 categorizes emergencies associated with systemic rheumatic diseases.¹

CLINICAL FEATURES AND DIAGNOSIS

Table 282-2 reviews clinical manifestations common to many of the systemic rheumatic diseases. Table 282-3 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. Complications of systemic rheumatic disease frequently require intensive care unit admission, and in 20% of patients with systemic rheumatic disease admitted to the intensive care unit, the diagnosis is made for the first time during the intensive care unit stay.² Rheumatoid arthritis is the most common rheumatic disease requiring intensive care unit admission, followed in decreasing frequency by systemic lupus erythematosus and systemic sclerosis.^2,3,4 Infection is the leading cause for intensive care unit admission, followed by rheumatic disease flare.