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

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.

Critical airway obstruction may develop (Table 282-4) at the level of the larynx, subglottic region, or trachea.

CRICOARYTENOID JOINT ARTHRITIS

In patients with rheumatoid arthritis, systemic lupus erythematosus, and relapsing polychondritis, arthritis or edema of the cricoarytenoid joints can lead to acute upper airway obstruction.⁵ In addition, secondary infections such as bacterial epiglottitis or tracheitis can quickly compromise the airway. Signs and symptoms of cricoarytenoid arthritis are throat pain or tenderness over cartilaginous structures (aggravated by swallowing or speaking), foreign body sensation or fullness in the throat, voice changes or hoarseness, and, in more severe cases, dyspnea, cough, or stridor.¹ Pain from cricoarytenoid arthritis is sometimes referred to the ear or to the neck.

CT scanning and fiber optic laryngoscopy can evaluate the cricoarytenoid joint. Initial treatment consists of systemic high-dose corticosteroids such as methylprednisolone 250 to 500 milligrams IV.⁵

TRACHEOMALACIA

Relapsing polychondritis can cause inflammation, destruction, and collapse of tracheobronchial cartilage, resulting in airway obstruction. Regions of segmental collapse due to tracheomalacia or refractory stenosis may be resected or treated with stents. The use of noninvasive ventilation may help to prevent airway collapse. Cartilaginous destruction results in a small glottis, so, when intubation is required, use a smaller endotracheal tube than normal.

SUBGLOTTIC STENOSIS

An acute upper airway obstruction can be observed in Wegener’s granulomatosis, resulting from subglottic stenosis with inability to clear tracheobronchial secretions. Subglottic stenosis can be a presenting sign of Wegener’s granulomatosis. Stenosis often requires surgical intervention.

INTUBATION IN PATIENTS WITH SYSTEMIC RHEUMATIC DISEASES

Endotracheal intubation is considered “difficult” in patients with systemic rheumatic diseases because of airway-related aspects of the disease. Anticipate the need for adjunctive airway techniques. Consider fiber optic intubation and fully prepare to perform an emergency cricothyrotomy. In cases where the airway is patent but intubation is anticipated and the patient can be transferred, it may be preferable to go to the operating room for a “double setup,” with preparation for a formal tracheostomy if oral intubation is unsuccessful.

Patients with rheumatoid arthritis and ankylosing spondylitis can develop temporomandibular joint dysfunction with reduced mouth opening. Patients also have a high incidence (25%) of atlantoaxial instability, C1-C2 subluxation, or dislocation. Therefore, avoid neck hyperextension in patients with rheumatoid arthritis.

Neck hyperextension and cervical manipulation are also a hazard in ankylosing spondylitis. Patients with cervical ankylosis are at high risk for cervical fractures (even after minor trauma).

In patients with scleroderma, hardening of the skin of the face and neck can be severe, decreasing the ability to open the mouth and limiting neck mobility.

Lung involvement, due to the underlying disease itself or secondary to infection, is a frequent cause of major morbidity and death (Table 282-4), particularly in systemic sclerosis, rheumatoid arthritis, systemic lupus erythematosus, Wegener’s granulomatosis, and polymyositis/dermatomyositis. Pulmonary complications of systemic rheumatic disease manifest primarily as interstitial lung disease and vascular disease. Due to chronic lung injury, interstitial pulmonary fibrosis, or pulmonary hypertension, even mild infection in patients with systemic rheumatic disease can cause respiratory failure.^6,7 Respiratory arrest has been reported in systemic lupus erythematosus and dermatomyositis/polymyositis patients due to phrenic nerve involvement, in rheumatoid arthritis patients due to cervicomedullary compression associated with rheumatoid atlantoaxial dislocation, and in Sjögren’s syndrome patients due to hypokalemic paralysis secondary to distal renal tubular acidosis.⁶

Symptoms can develop due to chronic manifestations of the underlying disease (i.e., interstitial lung disease), or new symptoms caused by an acute complication such as pneumonia or alveolar hemorrhage. Rheumatoid arthritis patients treated with methotrexate experience increased adverse respiratory events including pneumonia.⁸ Several complications outside the pulmonary parenchyma cause respiratory symptoms: involvement of the joints of the thoracic cage (in ankylosing spondylitis), pleural effusion, respiratory muscle inflammatory disease (polymyositis/dermatomyositis), cardiac involvement, and anemia. Pulmonary embolism risk is higher in systemic rheumatologic diseases.^9,10

ALVEOLAR HEMORRHAGE

Alveolar hemorrhage is an uncommon but catastrophic pulmonary emergency.^1,3 It is a complication of systemic lupus erythematosus (where it may be the presenting manifestation), antiphospholipid syndrome, systemic vasculitis, Wegener’s granulomatosis, dermatomyositis/polymyositis, microscopic polyangiitis, and systemic sclerosis. Early recognition and aggressive management are critical for improved outcome.^3,11 Delay in treatment, age >60 years old, end-stage renal failure, and cardiovascular comorbidity worsen prognosis.¹¹ Patients with alveolar hemorrhage complain of acute shortness of breath, fever, and cough. Symptom onset is usually abrupt, with a progression to respiratory failure requiring mechanical ventilation in more than half of cases.

The classical triad of hemoptysis, pulmonary infiltrates on chest x-ray, and rapid fall in hemoglobin level supports the diagnosis, but the triad is not always present.^12,13 New lung infiltrates (83% to 100%) and anemia (75% to 100%) are more sensitive signs of alveolar hemorrhage; the most common initial diagnosis is “atypical” pneumonia.¹² If symptoms are accompanied by high fever, it is difficult to distinguish between alveolar hemorrhage, acute lupus pneumonia, and infectious pneumonia. The distinction is of utmost importance, because the therapeutic options are exactly the opposite (immunosuppressant vs antibiotics). Emergency bronchoscopy with bronchoalveolar lavage can confirm the diagnosis but is done after admission. Treatment is directed to the underlying condition and includes high-dose glucocorticosteroids, cyclophosphamide, local vessel embolization, or plasma exchange.¹²

INTERSTITIAL LUNG DISEASE

Interstitial lung disease is characterized by infiltration of the pulmonary interstitium by inflammatory cells and matrix, leading to fibrosis, pulmonary hypertension, and respiratory insufficiency. Rheumatoid arthritis, systemic sclerosis, polymyositis/dermatomyositis, and Henoch-Schönlein purpura are commonly associated with interstitial lung disease. Interstitial lung disease may be asymptomatic, may produce slowly progressive symptoms (cough and dyspnea), or, rarely, can cause acute respiratory failure. Treatment in the ED is ventilatory support, with oxygen, noninvasive ventilation, and intubation as needed.¹⁴

PULMONARY HYPERTENSION

Pulmonary arterial hypertension can be a complication of any rheumatic disease with associated pulmonary fibrosis and interstitial lung disease, but is especially common in systemic sclerosis and systemic lupus erythematosus. Pulmonary vasculitis and pulmonary thromboembolism (i.e., in antiphospholipid syndrome patients) may also lead to pulmonary arterial hypertension. Clinical symptoms reported by patients with pulmonary arterial hypertension range from cough or mild shortness of breath, to severe dyspnea, cardiac arrhythmias, chest pain, and right ventricular failure. For further discussion, see chapters 57 and 58, “Systemic Hypertension” and “Pulmonary Hypertension,” respectively.

Heart disease develops through several pathophysiologic mechanisms, accounting for different manifestations: inflammation, fibrosis, infiltration, vasculitis, thromboembolism, and accelerated coronary atherosclerosis.¹⁵ Pulmonary hypertension can lead to right heart failure. Coronary atherosclerosis can be due to an imbalance between the inflammatory and anti-inflammatory activity and the use of specific drugs, such as corticosteroids. Table 282-5 provides a review of cardiac disorders in patients with systemic rheumatic diseases.^{15,16,17,18,19,20}