Michael P. Kefer
EPIDEMIOLOGY
Morbidity and mortality in rheumatic disease usually involves multiple organ systems and results from the disease, its complications, and/or its treatment (Table 183-1).
AIRWAY
Relapsing polychondritis presents with the abrupt onset of pain, redness, and swelling of the ears or nose. The tracheobronchial cartilage is involved in approximately 50% of cases. Hoarseness and throat tenderness over the cartilage are noted. Repeated attacks can lead to airway collapse.
Rheumatoid arthritis (RA) may involve the cricoary-tenoid joints causing pain with speaking, hoarseness, or stridor. The cricoarytenoid joints may fix in a closed position, which may mandate emergent trache-ostomy. Anticipate difficult endotracheal intubation from temporomandibular joint dysfunction, atlanto-axial instability, or cervical ankylosis.
TABLE 183-1 Common Features and Complications of Systemic Rheumatic Diseases
RESPIRATORY MUSCLE
Dermatomyositis and polymyositis may lead to respiratory failure from respiratory muscle involvement in poorly controlled disease.
LUNG
Pulmonary hemorrhage complicates Goodpasture’s disease, systemic lupus erythematosus (SLE), Wegener’s granulomatosis, and other vasculitic conditions.
Pulmonary fibrosis occurs in ankylosing spondylitis, scleroderma, and other conditions.
Pleural effusion occurs in RA and SLE.
HEART
Pericarditis occurs in RA and SLE.
Myocardial infarction may occur from coronary artery involvement in Kawasaki’s disease or polyarteritis nodosa.
Pancarditis occurs in acute rheumatic fever.
Valvular heart disease occurs in ankylosing spondylitis, relapsing polychondritis, and rheumatic fever. Involvement may extend into the conduction system causing arrhythmias.
NERVOUS SYSTEM
Patients with rheumatologic involvement of the cervical spine may be at high risk for cervical spine or spinal cord injury from otherwise trivial trauma as occurs with manipulation during endotracheal intubation if not done with extreme caution.
Destruction of the transverse ligament of C-2, with resultant symptoms of cord compression, may complicate RA.
Cervical spine inflexibility from ankylosing spondylitis predisposes to injury out of proportion to the mechanism.
Anterior spinal artery syndrome may result from rheumatologic conditions causing vasculitis, aortic dissection, or thromboembolism.
EYE
Temporal arteritis is a cause of sudden blindness and should be considered in any patient older than 50 years who presents with new-onset headache, visual change, or jaw claudication.
Dry eyes (and dry mouth) from Sjögren’s syndrome may occur alone or coexist with many rheumatologic conditions.
In RA, episcleritis is a self-limited, painless injection of the episcleral vessels. Scleritis presents as purple discoloration and marked tenderness of the eye with risk of scleral rupture.
KIDNEY
Nephritis is a common complication of SLE, Wegener’s granulomatosis, and systemic vasculitis.
Renal insufficiency can result from malignant hypertension as occurs with scleroderma, from rhabdomyolysis in the patient with florid myositis, or from prostaglandin inhibition by nonsteroidal anti-inflammatory drugs used in treatment.
Nephrotic syndrome in patients with SLE predisposes to renal vein thrombosis.
HYPERTENSION
Hypertension can complicate any condition that affects the kidneys directly, as in polyarteritis nodosa, scleroderma, or SLE, or indirectly, from nephrotoxic drugs used in treatment.
ADRENAL GLAND
Glucocorticoids are often used in the treatment of rheumatic conditions. Doses required may result in adrenal suppression. As a result, these patients are at risk for acute adrenal insufficiency.
BLOOD
Anemia and thrombocytopenia are common.
Many medications used for treatment are potent immunosuppressants.
For further reading in Tintinalli’s Emergency Medicine: A Comprehensive Study Guide, 7th ed., see Chapter 279, “Emergencies in Systemic Rheumatic Diseases,” by Gemma C. Morabito and Bruno Tartaglino.