6846 - Sarcoidosis

DBQ: Link to Index of DBQ/Exams by Disability for DC 6846

Acronym: SARC

Definition

A persistent disease that affects several systems. It is characterized by penetration of the affected organs by: T lymphoctyes, mononuclear phagocytes, and granulomas that alter the tissue structure.

Etiology

Generally, the disorder occurs from unknown etiology. However, it is stated that, a single aggravating agent or lack of defense reactions triggered by various insults may be responsible, and genetic factors may be important.

Signs & Symptoms

Symptoms depend on the system involved. Symptoms may be nonexistent, slight, or severe. Fever, weight loss, and arthralgias may be the initial symptoms. A constant fever is common if the liver is involved. Peripheral lymphadenopathy is common. The active granulomatous disease or secondary fibrosis may affect organ function. Cough and dyspnea may be slight or nonexistent. In addition, skin lesions, nasal and conjunctival mucosal granulomas and hepatic insufficiency may occur. Granulomas may cause inflammation of the uveal parts of the eye and, if untreated, may cause severe vision loss, severe vitreitis, or secondary glaucoma. Heart involvement may cause angina, heart failure, or fatal conduction irregularity. Moreover, acute polyarthritis may be an outstanding symptom. Chronic periarticular swelling and tenderness may occur. The most prominent central nervous system (CNS) symptom is cranial nerve palsies. Finally, diabetes insipidus, and hypercalcemia and hypercalciuria may cause renal calculi or nephrocalcinosis with resulting renal failure.

Tests

Ninety percent of patients with sarcoidosis have an irregular chest x-ray. Laboratory studies reveal: leukopenia, serum uric acid elevation, and serum alkaline phosphatase and glutamyl transpeptidase levels may be elevated due to liver involvement. In addition, hypergammaglobulinemia occurs frequently in African Americans.

A series of pulmonary function tests are necessary to evaluate the disease and guide treatment. Tissue biopsy is essential to differentiate the disease from other diagnoses, e.g., lymphoma and fungal infections. In addition, fiberoptic bronchoscopy and thoracoscopy may be instituted. Finally, total body gallium scanning may be used for diagnosis in patients with normal chest x-rays or otherwise unusual presentations.

Treatment

Patients with minimal or no symptoms are not to be treated unless they are experiencing sustained hypercalcemia. There are no drugs noted that consistently prevent pulmonary fibrosis. Corticosteroids should be given to lessen the effects of severe symptoms, e.g., dyspnea, arthralgia or fever. Corticosteroids are also indicated if hepatic, cardiac, CNS, or ocular effects exist, and if disfiguring lesions or hypercalcemia are evident.

However, about 10% of patients are unresponsive to corticosteroids, and are usually given methotrexate. Finally, patients experiencing acute disease (e.g., severe erythema nodosum) may require treatment for a few weeks, but most of those requiring therapy have chronic sarcoidosis and require treatment for more than one year.

Residuals

It is noted that approximately 10% of patients acquire serious disability from organ damage. However, mortality from sarcoidosis is less than 3%.

Special Considerations

  • May be entitled to special monthly compensation where the Veteran has a single service-connected disability rated as 100% with additional service-connected disability or disabilities independently ratable at 60% or more, which are separate and distinct from the 100% service-connected disability and involves different anatomical segments or bodily systems. See  38 CFR 3.350(i)(1) – Special Monthly Compensation (SMC).

  • This disease shall be granted service connection although not otherwise established as incurred in or aggravated by service if manifested to a compensable degree within the applicable time limits under 38 CFR 3.307 following service in a period of war or following peacetime service on or after January 1, 1947, provided the rebuttable presumption provisions of 38 CFR 3.307 are also satisfied [38 CFR 3.309 [Disease subject to presumptive service connection], 38 CFR 3.309(a). [chronic disease].

Notes

  • Review special provisions regarding the evaluation of specific respiratory conditions under 38 CFR 4.96(a) - Rating co-existing respiratory conditions.