The clinical manifestations of SLE are varied and any organ can be affected. Severity varies from mild to severe and typically there are periods of disease flares interspersed with stability (1). Potential manifestations are (1,2,3):

Treatment of SLE involves immunosuppressive and anti-inflammatory medications. Topical or low-dose oral corticosteroids, NSAIDs, and antimalarial medications are used for mild cases (1). High-dose corticosteroids, alkylating
agents (cyclophosphamide), purine synthesis inhibitors (azathioprine, mycophenolate mofetil), methotrexate, intravenous gamma globulin, plasmapheresis, and biologic therapies (belimumab, rituximab, monoclonal antibodies) are used when there is significant organ involvement (1). Aggressive medical treatment is recommended for patients with hypertension or thrombophilia (1). Mortality in patients with SLE is bimodal with early death caused by either active lupus or severe infections from immunosuppressants. Late death is caused by cardiovascular disease from the atherosclerotic effects of SLE and chronic steroid use (4). Males and patients with later-onset-age disease tend to have a worse prognosis (2).

Since both active lupus and chronic steroids affect the musculoskeletal system (avascular necrosis, osteoarthritis, osteoporosis, and vertebral fractures), many patients undergo orthopedic procedures (5). There is an increased risk of perioperative complications and mortality in patients with SLE undergoing hip arthroplasty, but not knee arthroplasty (5). Complications include increased transfusions, hematomas, thrombosis, infections, aseptic loosening, prosthesis dislocation, longer hospital stay, falls, deep vein thrombosis, AKI, superficial wound infections, and requiring revisions (5). SLE patients may present for kidney transplantation or cardiovascular procedures.

Depending on the extent of the tissue damage and complications of treatment, patients with SLE have high rates of perioperative morbidity and mortality. The stress of surgery can cause disease flares. Data from a national database in Taiwan (4) showed that the odds ratio (OR) of 30-day mortality for patients with SLE having major surgery was 1.71 compared to patients without SLE. Hospitalization in the 6 months before a major surgery increased the OR of developing AKI, pulmonary embolism, stroke, sepsis, and 30-day mortality. The authors proposed that prior hospitalization, especially for the purpose of giving a steroid burst, is a marker for the severity of the patient’s disease (4).

Preoperative assessment includes evaluating the extent of tissue involvement and the degree of organ damage especially in regards to the airway (laryngeal involvement, cervical subluxation), pulmonary, cardiovascular, renal, hematologic, and neuropsychiatric systems. Preoperative tests such as CBC, BMP, and ECG should be obtained. Based on the history and physical examination and the surgery planned, other tests may be indicated such as chest radiography (CXR), liver panel, coagulation studies, cardiac workup (echocardiogram, stress test), and cervical neck evaluation (neck flexion/extension or MRI). Identification of patients with APS is important due to an overall higher risk of arterial and VTE (6). Consultation with the patient’s rheumatologist to discuss the status of disease and management of immunosuppressive and anticoagulant medications should be considered. A careful assessment of medications is necessary with a determination whether to discontinue immunosuppressive agents (see Table 10.1) or anticoagulants (with bridging therapy if indicated based on the risk of thrombosis) (7). Patients may need additional steroids perioperatively to prevent acute adrenal insufficiency. Elective surgery is delayed during a flare or if there is an acute infectious process.

Sarcoidosis can affect any organ. Signs and symptoms are nonspecific including the presence of tissue granulomas (1). The lungs, eyes, skin, and lymphatic system are most frequently affected (2). The disease can be acute, subacute, chronic, or progressive (6). Up to 50% of patients present with asymptomatic bilateral hilar adenopathy found on CXR (1). Other classic presentations include Löfgren syndrome (bihilar lymphadenopathy, bilateral arthritis of the ankles, erythema nodosum, and general flu-like symptoms) and Heerfordt syndrome (uveitis, parotitis, Bell palsy, and fever) (2). Ultimately, 90% of patients will have pulmonary manifestations and an abnormal CXR (2). High-resolution computed tomography (CT) is more sensitive for detection of adenopathy and subtle parenchymal disease (5). Pulmonary fibrosis and pulmonary hypertension are independent risk factors for mortality (6). The frequency
of airway involvement increases as pulmonary parenchymal disease progresses and is associated with increased morbidity and mortality (7). Airway effects include mucosal erythema, edema, hoarseness, dysphagia, laryngeal paralysis, airway obstruction, obstructive sleep apnea (OSA), airway hyperreactivity, bronchiectasis, bronchiolitis, and external compression (7). Symptoms of sarcoidosis include persistent cough, fever, arthralgias, visual changes, skin lesions, dyspnea on exertion, or simply fatigue (1,2). Cardiac symptoms occur in 5% of patients, although actual involvement of the heart is close to 25% (2). Cardiac manifestations include high-degree atrioventricular (AV) block, ventricular tachycardias, and cardiomyopathy (2). Nervous system manifestations include cranial neuropathy (especially, cranial nerve VII), involvement of the hypothalamus and pituitary gland (leading to hypoglycemia, impaired temperature regulation, DI, SIADH), papillary edema, increased intracranial pressure, hydrocephalus, aseptic meningitis, intracerebral lesions, seizures, spinal lesions, or psychiatric symptoms (2,8). Peripheral neuropathy can cause autonomic dysfunction, hypoesthesia, paresthesia, neuropathic pain, restless leg syndrome, and sleep disturbances (2). Hepatic involvement occurs in 70% of patients with sarcoidosis, but most are asymptomatic with elevated liver enzymes (3). Symptoms include abdominal pain, fatigue, pruritus, and jaundice. Chronic hepatic involvement can potentially lead to portal hypertension and cirrhosis (3). Esophageal involvement is not common. Symptoms can include dysphagia from muscle infiltration, enteric nervous plexus neuropathy, and external compression (9). The kidney can be affected by hypercalcemia and hypercalciuria that can occur with sarcoidosis or by granulomatous interstitial nephritis. Rarely, renal failure occurs (10).

Patients with sarcoidosis may present for diagnosis (biopsy of skin or lymph nodes, bronchoscopy, endobronchial ultrasonography-guided transbronchial biopsy), treatment of airway obstruction, or treatment of organ damage (procedures for treating arrhythmias, organ transplantation) (2,6). Significant involvement of the airway (obstruction) or vital organs (pulmonary fibrosis, pulmonary hypertension, arrhythmias, cardiomyopathy,
cirrhosis, and chronic kidney disease) increase perioperative morbidity and mortality. Survival of patients with sarcoidosis after transplantation is similar to patients who do not have sarcoidosis (6).

It is important to assess the affected organs and the extent of damage especially in regards to the airway, lungs, heart, liver, kidneys, central, and peripheral nervous systems. Patients should be screened for OSA. Depending on the history and physical exam, further testing such as ECG, echocardiogram, CBC, and serum creatinine may need to be performed preoperatively. Vital capacity, as measured on pulmonary function tests, is an important parameter for monitoring the course of the disease (2). Elective surgery is delayed until assessment is complete and the disease is optimally managed. If a patient has been treated with steroids, a steroid bolus perioperatively may be needed to prevent acute adrenal insufficiency.

Careful airway assessment is mandatory. Severity of reflux symptoms are ascertained. Preoperative testing is shown in Table 10.3.

Antireflux medications, vasospasm, and pulmonary hypertension treatments are continued through the perioperative period. For management of immunosuppressives, see Table 10.2.

Hemoglobin should be optimized, especially before major surgeries. It is recommended to keep patient care areas at thermoneutral temperature of about 20°C to limit Raynaud symptoms. Pulse oximetry may be hampered by Raynaud’s, an ear

probe may give more reliable results. Arterial line placement carries higher than usual risk due to already poor circulation. VTE is three times more common in this population and appropriate prophylaxis is necessary (6).