Sarcoidosis

At a glance

An inflammatory disease characterized by the presence of granulomas (small areas of inflamed cells) that usually affects the lungs. Pulmonary sarcoidosis usually leads to restrictive pulmonary disease with significant loss of lung tissue. However, all organs can be affected and the nodule can be seen as sores if the skin is involved.

Synonyms

Boeck Sarcoid; Besnier-Boeck-Schaumann Disease; Schaumann Disease.

Nature

May have a nongenetic basis but presents a familial predisposition. Seems to be more likely to present with class I HLA A1, B8, and DR3.

Incidence

Actual incidence unknown. Affecting persons of all races, both sexes, and all ages worldwide. Interestingly, cases present most commonly in the winter and early spring months. About 1 to 40 cases per 1,000,000 population. More frequent in blacks than in whites.

Diagnosis

Systemic disorder of unknown cause characterized by noncaseating granulomas often diagnosis of exclusion. Most typically, sarcoidosis is an interstitial lung disorder involving alveoli, blood vessels, and bronchioles, and producing clinical findings of dry rales, restricted lung volumes, and abnormalities in gas exchange.

Clinical aspects

Bilateral hilar adenopathy, pulmonary infiltration. Erythema nodosum, maculopapular rash, enlarged lacrimal glands, iridocyclitis, glaucoma, and chorioretinitis. Enlarged salivary glands. Bone cysts. Associated with inflammatory bowel disease, especially Crohn disease. Hyperglobulinemia, multisystem granulomatous disease with positive Kveim test. Neurosarcoidosis can mimic more common disease processes, such as meningioma, glioma, or metastases. The granulomas are frequently present in multiple organs, including the liver. Serious cardiac dysfunction is detected in 5 to 10% of patients. Sudden deaths are described. The main clinical feature with cardiac sarcoidosis is the recording of ECG abnormalities in terms of rhythm, conduction, and repolarization. Some patients have papillary muscle dysfunction, infiltrative cardiomyopathy with congestive heart failure, and pericarditis.

Precautions before anesthesia

It is highly recommended to obtain an anesthesiology consultation before any elective surgical procedures. Because all organs can be involved, perform a complete cell blood count, electrolytes (sodium, potassium and calcium), creatinine, liver function tests, ECG, and chest radiography. In an acute case, echocardiography can be helpful. The serial pulmonary function tests and chest radiography are the best predictive values in long term.

Anesthetic considerations

Airway complications caused by laryngeal involvement and bronchial stenosis have also been described. Avoid the use of bougies if endobronchial sarcoidosis is suspected.

Pharmacological implications

Because these patients are chronically on steroid therapy, they will need to receive steroid supplementation during the perioperative stress period. Sometimes the musculoskeletal involvement responds better to methotrexate. Avoid succinylcholine and other drugs that may increase intraocular pressure in the presence of glaucoma.

References