RISK FACTORS : CHR USE OF STEROID MAY INCREASE THE RATE OF VANTRICULAR ANEURYSM. VENTRICULAR ANEURYSMS INCREASE THE RISK OF VENTRICULAR TACHYCARDIA
PRESENTATION OF DISEASE DEPENDS ON THE ORGAN INVOLVED :
* - PULMONARY
* - DERMATOLOGICAL
* - CARDIAC : PACE MAKER, HEART FAILURE MEDICINES, DEFIBRILLATOR, ANTIARRHYTHMIC MEDICINES, ACCORDING TO PRESENTATION.
* - OCULAR
* - NEUROLOGICAL
TT FOR RESTRICTIVE LUNG INVOLVEMENT :
Medical Care: Few reliable studies on disease indications and optimal treatment exist. Most patients (>75%) require only symptomatic therapy (nonsteroidal anti-inflammatory drugs). Approximately 10% of patients need treatment for extrapulmonary disease, while 15% of patients require treatment for persistent pulmonary disease.
" Corticosteroids are the mainstay of therapy.
o Generally, prednisone given daily and then tapered over a 6-month course is adequate for pulmonary disease. Earlier recommendation suggested an initial dose of 1 mg/kg/d of prednisone; however, more recent expert opinions endorse a lower dose (eg 40 mg/d), which is tapered to every other day long-term therapy over several weeks. Most patients who require long-term steroids can be treated using 10-15 mg of prednisone every other day.
o Recent data suggest that corticosteroid use may be associated with increased relapse rates.
o High-dose inhaled corticosteroids may be an option, but conclusive data are lacking.
" Noncorticosteroid agents are being used more frequently.
o Methotrexate has been a successful alternative to prednisone and is a steroid-sparing agent.
o Hydroxychloroquine may be used for cutaneous lesions, hypercalcemia, neurological sarcoidosis, and bone lesions.
o Other alternatives include cyclosporine, pentoxifylline, and azathioprine.
" Infliximab and thalidomide have been used for refractory sarcoidosis, particularly for cutaneous disease. Infliximab appears to be an effective treatment for patients with systemic manifestations such as lupus pernio, uveitis, hepatic sarcoidosis, and neurosarcoidosis.
" Tetracyclines have shown promise for the treatment of cutaneous sarcoidosis.
" For pulmonary disease, asymptomatic PFT and/or CXR abnormalities are not an indication for treatment. In patients with minimal symptoms, serial reevaluation is prudent. Significant respiratory symptoms associated with PFT and CXR abnormalities likely require therapy. For such patients, treatment is indicated if objective evidence of recent deterioration in lung function exists. Corticosteroids can result in small improvements in the functional vital capacity, and in radiographic appearance in patients with more severe stage II and III disease.
" One recent study demonstrated an approach that may minimize the use of corticosteroids without harming the patient. This is accomplished by withholding therapy unless the patient shows at least a 15% decline in one spirometric measure associated with increasing symptoms or, if asymptomatic, withholding therapy unless the patient shows worsening PFTs and a change in CXR.
" For extrapulmonary sarcoidosis involving such critical organs as the heart, liver, eyes, kidneys or central nervous system, corticosteroid therapy is indicated.
" Topical corticosteroids are effective for ocular disease.
" Inhaled corticosteroids are occasionally used, in particular in patients with endobronchial disease.
Surgical Care: For patients with advanced sarcoid-induced pulmonary fibrosis, lung transplantation remains the only hope for long-term survival.
" Lung transplantation is a viable option for patients with stage IV sarcoidosis. Transplantation in such patients should be strongly considered when the forced vital capacity (FVC) falls below 50% predicted and/or the forced expiratory volume in 1 second (FEV1) falls below 40% predicted.