General discussion

SARCOIDOSIS. Sarcoidosis is a multi system inflammatory disorder of unknown etiology that predominantly affects the lungs and intra thoracic lymph nodes. Sarcoidosis is manifested by the presence of non caseating granulomas ( NCG's ) in affected organs. It is characterised by a seemingly exaggerated immune response against a difficult - to - discern antigen. SIGNS AND SYMPTOMS. The presentation of sarcoidosis depends on the extent and severity of organ involvement. *Asymptomatic. *Systematic complaints like fever and anorexia. *PULMONARY MANIFESTATION Dyspnea on exertion. Cough. Chest pain Hemoptysis. Pulmonary findings on physical examination can be Usually normal. Crepitus. External oxygen desaturation. LOFGREN SYNDROME. Fever,bilateral hilarious lymphadenopathy and polyarthralgias. DERMATOLOGICAL MANIFESTATION. *-Erythema nodosum. *A lower extremity panniculitis with painful erythematous nodules. *Lupus permit ( the most specific associated cutaneous lesion ) *Violaceous rash on cheeks and nose ( common ) *Maculopapular plaques ( uncommon ) OCULAR MANIFESTATION. *Anterior or posterior granulomatous uveitis. *Conjunctival lesions and scleral plaques. If untreated can lead to blindness. OTHER POSSIBLE MANIFESTATION. *Osseous involvement. *Heart failure from cardiomyopathy. *Heart block and sudden death. *Lymphocytic meningitis. *Cranial nerve palsies and hypothalamic / pituitary dysfunction. DIAGNOSIS. *Chest X-RAY central to the evaluation. *High resolution CT identifies active alveolitis versus fibrosis. *Gallium scans. *Pulmonary function tests and carbon monoxide diffusion capacity test of the lungs( DLCO ) for carbon monoxide is used routinely in evaluation and follow up. An isolated decrease in DLCO is the most common abnormality. *Cardiopulmonary exercise testing is a sensitive test for identifying and quantifying the extent of pulmonary involvement.I t also suggests cardiac involvement that otherwise is not evident. IMPAIRED HEART RATE RECOVERY DURING THE FIRST MINUTE FOLLOWING EXERCISE HAS BEEN SHOWN TO BE AN INDEPENDENT PREDICTOR FOR CARDIOVASCULAR AND ALL CAUSE MORTALITY. DIAGNOSIS REQUIRES BIOPSY IN MOST CASES.ENDOBRONCHIAL BIOPSY VIA BRONCHOSCOPY IS OFTEN DONE.THE CENTRAL HISTOLOGICAL FINDING IS THE PRESENCE OF NON CASEATING GRANULOMAS WITH SPECIAL STAINS NEGATIVE FOR FUNGUS AND MYCOBACTERIA. *Hypercalcemia. *Hypercalcuria. *Elevated alkaline phosphatase level. *Elevated angiotensin converting enzyme level. STAGING OF SARCOIDOSIS. STAGE O : Normal chest radiographic findings. STAGE I. : Bilateral hilar lymphadenopathy. STAGE II : Bilateral hilar lymphadenopathy and infiltrates. STAGE III : Infiltrates alone. STAGE IV : fibrosis. TREATMENT. Most patients do not require therapy and their condition improves spontaneously. Markers for poor prognosis are Advanced chest radiography stage. Extra pulmonary disease Evidence of pulmonary hypertension. Most patients require symptomatic treatment with NSAID 's for treatment of arthralgias. Treatment for patients with pulmonary involvement. *Asymptomatic patients do not require treatment. *In patients with minimal symptoms,serial re evaluation is prudent *Treatment is indicated for patients with severe symptoms. *Corticosteroid are helpful. *For extra pulmonary sarcoidosis involving heart,liver,eyes,kidney and central nervous system , corticosteroid therapy is indicated. *Topical steroids are useful for ocular disease. Common indications for non - corticosteroid are *Steroid resistant disease. *Intolerable adverse effects of steroids. Non corticosteroid agents are METHOTREXATE CHLOROQUINE and HYDROCHLOROQUINE used for cutaneous lesion,hypercalcemia,neurologic sarcoidosis and bone lesions. CHLOROQUINE is useful for Acute and maintenance treatment of chronic pulmonary sarcoidosis. CYCLOPHOSPHAMIDE is used in refractory sarcoidosis. AZATHIOPRINE is best used as a steroid sparing agent. CHLORAMBUCIL is beneficial in patients with progressive disease unresponsive to steroids. CYCLOSPORINE is of limited benefit in skin sarcoidosis or in progressive sarcoidosis resistant to conventional therapy. INFLIXIMAB & THALIDOMIDE are used for refractory sarcoidosis. FOR PATIENTS WITH ADVANCED PULMONARY FIBROSIS FROM SARCOIDOSIS,LUNG TRANSPLANTATION REMAINS THE ONLY HOPE FOR LONG TERM SURVIVAL. LONG TERM MONITORING. *Monitor pulmonary function and chest radiography every 6 months. *Assess for progression or resolution. *Determine if previously uninvolved organs have become affected. *Annual slit lamp examination and ECG are recommended.

The most serious long term side effect of bleomycin therapy is 1) Pulmonary fibrosis 2) Cataract formation 3) Cardiomyopathy 4) Aplastic anemia

a 27 yr female , SOB grade 4 since 5 days,her hb is 6.7gms ,b/l pedal. edema, 2d echo was EF 45% ,RF positive, ESR 70 1st hour ,now she is on O2 inhalation through mask 3 lit,inj piptaz 4.5 gr and inj moxiflox,inj dexamethasone,inj pan 40, any modifications regarding medication and management, advised 2units PRBC,pleural taping was unsuccessful,??? thankyou

64 year old male with complaints of episodic sweating for the last 4 months.Occasional dry cough.No ho fever or weight loss.Non-smoker.Hb 14,wc 14400,esr 35,FBS 114,T3T4TSH normal.

35y male known htn complaints of recurrent low grade fever loss of weight and appetite pain around umbilicus since 2month.Recently investigated for cbc ESR HIV hbsag with normal findings.Mauntox test is significant (30mm ) suggest dd n tr?