English

Abstract

Sarcoidosis is a granulomatous lesion affecting multi organs, mainly the lungs, kidney, skin, lymph nodes etc. occurring mainly in African American blacks and Nordics. A 45 year old female presented with progressively increasing generalised skin eruption, fatigue and joint pains. Further work up of the patient reveals Hypothyroidism, Rheumatoid factor positive and Lipid profile showed isolated hypertriglyceridemia. CXR-PAV showed bilateral hilar lymphadenopathy. Calcium profile was normal. Bone marrow examination for anemia showed non-caseating well formed epithelioid granuloma strongly suggestive of Sarcoidosis. High ACE level of 114 clinched the diagnosis for Sarcoidosis. The patient responded to steroid and is on remission. INTRODUCTION: Sarcoidosis was first described by Jonathan Hutchinson in 1869. Sarcoidosis is literally taken from sarc meaning flesh, -oid, like, and -osis, diseased or abnormal condition, is also called sarcoid, Besnier-Boeck disease or Besnier-Boeck-Schaumann disease, is a disease in which abnormal collections of chronic inflammatory cells (granulomas) form as nodules in multiple organs.(1) The cause of sarcoidosis is unknown. Granulomas of non-caseating type most often appear in the lungs or the lymph nodes, but virtually any organ can be affected. Normally the onset is gradual. Sarcoidosis may be asymptomatic or chronic. It commonly improves or clears up spontaneously. Sarcoidosis most commonly affects young adults of both sexes, although studies have reported more cases in females. Incidence is highest for individuals younger than 40 and peaks in the age-group from 20 to 29 years; a second peak is observed for women over 50.(2)(3) Sarcoidosis occurs throughout the world in all races with an average incidence of 16.5/100,000 in men and 19/100,000 in women. The disease is most prevalent in Northern European countries, and the highest annual incidence of 60/100,000 is found in Sweden and Iceland. In the United States, sarcoidosis is more common in people of African descent than Caucasians, with annual incidence reported as 35.5 and 10.9/100,000, respectively. (4) Granulomatous inflammation is characterized primarily by accumulation of monocytes, macrophages and activated T-lymphocytes, with increased production of key inflammatory mediators, TNF-alpha, IFN-gamma, and IL-12, characteristic of a Th1-polarized response (T-helper lymphocyte-1 response). Sarcoidosis has paradoxical effects on inflammatory processes; it is characterized by increased macrophage and CD4 helper T-cell activation resulting in accelerated inflammation, however, immune response to antigen challenges such as tuberculin is suppressed. This paradoxic state of simultaneous hyper- and hypo- activity is suggestive of a state of anergy. The anergy may also be responsible for the increased risk of infections and cancer. It appears that