Sarcoidosis is inflammatory illness affecting many different organs and organ systems. In most cases inflammation is first noticed in the lungs and intrathoracic lymph nodes. The very condition is also characterized by formation of noncaseating granulomas in the tissues affected by inflammation.
In spite of all efforts scientists have made, sarcoidosis remains the disease of unknown cause. Still, all the symptoms and signs as well as complications of the disease are easily explained by inflammatory reaction of the affected organs and formation of characteristic granulomas.
It is, however, shown that T cells play significant, if not crucial, role in the development of the disease. These cells are responsible for propagation of excessive cellular immune reaction. Furthermore, patients suffering from sarcoidosis have increased amount of TH 1 cytokines (interferon). Finally, the amount of tumor necrosis factors as well as the number of its receptors is much greater in such patients comparing to healthy individuals. All the mentioned drives to conclusion that the immune system plays significant role in the onset and further progression of the disease.
Sarcoidosis Symptoms and Signs
Presentation of sarcoidosis generally depends on the affected organ and the severity of inflammation. It is estimated that around 5% of all cases are diagnosed incidentally when chest X-ray is performed in other purposes.
Around half of all patients have to deal with systemic complaints like fever, anorexia and arthralgia.
Pulmonary sarcoidosis is characterized by dyspnea on exertion, chest pain, cough and hemoptysis (coughing up blood). In end stage of pulmonary sarcoidosis the lungs are affected by severe fibrosis and symptoms and signs of the disease are quite severe.
In people in whom the skin gets affected clinical findings include erythema nodosum, lupus pernio, violaceous rash, osseous involvement, maculopapular plaques etc.
Ocular manifestations develop in a form of anterior/posterior granulomatous uveitis, conjunctival lesions and scleral plaques etc. If left untreated, some eye problems may eventually cause blindness.
If the heart is affected, one may eventually develop heart failure which usually results from cardiomyopathy. Heart block and sudden death may occur as well.
Finally, neurological manifestations in people suffering from sarcoidosis include cranial nerve palsies, hypothalamic/pituitary dysfunction and lymphocytic meningitis.
The condition is treated with potent anti-inflammatory drugs. The cornerstones of the therapy are corticosteroids. Apart from corticosteroids patients may also be prescribed methotrexate, chloroquine and hydroxychloroquine, cyclophosphamide, azathioprine, chlorambucil, cyclosporin and infliximab. Which of the mentioned drugs is going to be prescribed depends on that affected organ/organ system and the severity of the condition.
Unfortunately, advanced stage of the disease, especially advanced pulmonary fibrosis is life-threatening and inevitably leads to death unless patients undergo lung transplantation.