Acute cutaneous lupus (e.g., malar rash)
Chronic cutaneous lupus (e.g., classic discoid rash)
Oral or nasal ulcers
Joint disease—either synovitis or tenderness in ≥2 joints
Serositis—either pleural (pleurisy, effusion, or rub) or pericardial (pain, effusion, rub)
Renal—either proteinuria or red blood cell casts
Neurologic—seizures, psychosis, neuritis, myelitis, peripheral/cranial neuropathy, acute confusional state
Leukopenia or lymphopenia
Elevated antinuclear antibody (ANA) level
Elevated anti-double-stranded DNA level
Presence of anti-smooth muscle antibody
Antiphospholipid antibody positive (positive lupus anticoagulant, medium/high titer anticardiolipin, positive anti-beta-2 glycoprotein 1)
Positive direct Coombs test in the absence of hemolytic anemia
General—fatigue, malaise, weight loss, fevers
Dermatologic—rashes, photosensitivity, mucositis, oral, nasal or genital ulcers, nonscarring alopecia
Musculoskeletal—arthralgias, myalgias, arthritis, myositis, osteoporosis, atlantoaxial subluxation
Renal—nephritis, proteinuria, hematuria, nephrotic syndrome, end-stage renal disease (ESRD)
Hematologic/immunologic—autoantibodies, hemolytic anemia, thrombocytopenia, leukopenia, lymphopenia, lymphadenopathy, splenomegaly, hematologic malignancies
Respiratory—pleurisy, pleural effusion, pneumonitis, interstitial lung disease, pulmonary hypertension, pulmonary alveolar hemorrhage, vocal cord paralysis, subglottic stenosis, laryngeal edema, epiglottitis, rheumatoid nodules, inflammatory masses, cricoarytenoiditis
Cardiovascular—vasculitis, Raynaud’s, thromboembolic disease, premature atherosclerosis, carotid vascular disease, pericarditis, pericardial effusion, pericardial tamponade, valvular disease, myocarditis, arrhythmias, coronary artery disease, myocardial infarction, cardiomyopathy, Libman-Sacks endocarditis
Neuropsychiatric—headaches, cognitive dysfunction, psychosis, acute confusional state, neuritis, peripheral/cranial neuropathy, myopathy, movement disorder, transverse myelitis, seizures, cerebrovascular disease, white matter lesions, cerebral infarction, venous sinus thrombosis, atrophy
Gastrointestinal—abdominal pain, elevated liver enzymes, pancreatitis, esophagitis, bowel necrosis from mesenteric vasculitis/ischemia
Pregnancy—increased risk of miscarriage and poor fetal outcome
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).
TABLE 10.1 Usual Recommended Perioperative Medication Management
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).
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).
Localized scleroderma (morphea) of which there are several types based on severity and distribution
Involvement of the skin of the face, trunk, and distal limbs
Sclerodactyly—sausage-shaped fingers (and toes) that are erythematous, swollen, and shiny due to tightening of the skin are common
Skin fibrosis can be complicated by necrosis
Limited cutaneous systemic sclerosis
Usually a combination of scleroderma features
CREST syndrome—calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia
Pulmonary involvement includes interstitial lung disease and pulmonary hypertension. Pulmonary hypertension is associated with poorer overall survival rates (2).
Diffuse cutaneous systemic sclerosis
Rapidly progressive disease, with generalized skin involvement and cardiovascular complications with vascular involvement, coronary artery disease, cardiomyopathy, and hypertension
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).
TABLE 10.2 Immunosuppressive Therapies for Scleroderma/Recommendations for Holding Perioperatively
TABLE 10.3 Preoperative Testing
Constitutional features such as fever, weight loss, malaise, and anemia of chronic disease (normochromic, normocytic)