Pulmonary hypertension (PH) is hemodynamically defined as a mean pulmonary artery pressure (mPAP) >20 mm Hg at rest, measured by right heart catheterization. The American Heart Association and the European Society of Cardiology/European Respiratory Society guidelines recognize this threshold for all PH groups. The severity of PH is often further stratified based on mPAP or, in echocardiographic practice, by estimated right ventricular systolic pressure (RVSP) or systolic pulmonary artery pressure (sPAP).[1-4]
There is no universally accepted, guideline-based classification of PH severity as "mild," "moderate," or "severe" using mPAP or RVSP cutoffs in the most recent consensus documents. However, commonly used clinical thresholds, supported by the American Society of Echocardiography, are:
• Mild PH: RVSP or sPAP 36–49 mm Hg
• Moderate PH: RVSP or sPAP 50–59 mm Hg
• Severe PH: RVSP or sPAP ≥60 mm Hg
These severity categories do not distinguish between the five clinical groups of PH (e.g., PAH, PH due to left heart disease, etc.), which are defined by etiology and additional hemodynamic parameters (e.g., pulmonary artery wedge pressure, pulmonary vascular resistance) as outlined by the World Symposium on Pulmonary Hypertension and the American Heart Association.
These categories are adjuncts to, not replacements for, the hemodynamic and etiologic classification of PH, which remains essential for diagnosis and management.
SArcoidosis
Pathophys - inflammation produces tiny lumps of cells in organs throughout the body (granulomas) occur in lungs, LN, eyes, skin, other areas
Pulmonary sarcoid - reduce amount of air lungs can hold - abnml stiffness -> breathing problems
20-40 years of age
symptoms to ask - wt loss
Pulm - SOB / dyspnea
LN - enlarged LN
Eyes - blurred vision
Bones / Joints - nodules swelling
spleen / liver - pain under abdomen under ribs
Heart - irregular fasat heart beats, palpitations, sudden cardiac death, arrhythmias
Salivary glands - dry mouth / throat
Nervous system - bells palsy, vision issues, wkness / numbness of arm / leg, drooping of one side of face, pins / needles symptoms
Diagnosis
- CXR enlarged L.N / round spots, PFTs, Tissue biopsy, BAL, eyeexam, blood tests, CT scan, GAllium scan active sarcoidosis in many organs, EKG
Treatment - corticosteroids, methotrexate, azathioprine, hydroxychloroquine, chlorambucil, cyclophosaphamide, pentoxifylline, thalidomide, minocycline, infliximab
Remidcade - infliximab
remicade - side effects - h/a, nausea, itching, stinging, dizziness, fatigue, infusion related reactions, can develop antibodies to remicade.
What is Remicade?
Remicade is an entirely new type of drug, it is an antibody that blocks a substance called TNF. (TNF stands for "Tumor Necrosis Factor" - but this has nothing to do with tumors or cancer.) This newly discovered protein has been found in the blood of patients with active inflammation like Crohn's disease and Rheumatoid Arthritis.
A biological agent is a "designer molecule" made to hit a specific target. Remicade is a new biological agent that was made to target only TNF. It is co-produced in mice and the final product is about 75% human and 25% mouse antibody. " Remicade was approved by the FDA in May 1998 for treatment of Crohn's disease. It was recently also approved to treat patients with Rheumatoid Arthritis whom also seem to have an excess amount of TNF in their joints. It is used for sarcoidosis.
How is Remicade administered?
Remicade is given as a solution by intravenous infusion (into a vein). This infusion may be given in your doctor's office, or at an infusion center, or in an outpatient setting your doctor selects. The actual infusion takes 2 hours. You should plan on spending about 3 hours, which includes preparation. During this time, the nurse will check on you at regular intervals. The infusion is not sedating and most patients are able to drive home.
What are the side effects?
In medical studies, most people had no side effects related to Remicade. Remicade infusions are usually well tolerated. Immediate side effects occur in about 5% of patients and include headache, nausea, itching, stinging, dizziness, fatigue, and fever and did not last long. Only 2% of people had to stop the infusion because of side effects. The infusion-related reactions, if they occurred, were typically mild, requiring reduction of infusion rate, administration of acetaminophen, and/or Benedryl. Pretreatment with Tylenol and Benedryl may be of benefit. Serious infusion-related reactions are uncommon, but can occur. That is why it is best to have the infusion in a controlled safe environment.
Some patients develop antibodies to Remicade that may result, rarely, in the development of a lupus-like syndrome. Recent data show this to occur in less than 1% of patients. Symptoms included migratory joint pain, pleuritis, and rash. There were no kidney or central nervous system symptoms. These symptoms resolved by simply stopping treatment and a short couse of steroids.
Rarely serious side effects may occur. Since this treatment partially blocks your immune system, patients treated with Remicade may have an increased risk of infection. The infections most frequently reported were upper respiratory tract infections (including, sinusitis, pharyngitis, and bronchitis) and urinary tract infections. Rarely, serious infections have been reported in patients, some fatal. You should not take Remicade if you have an acute or chronic infection.
Can Remicade give me cancer?
In the short term, no. Despite some intitial concerns, recent studies show no statistically significant increased incidence of cancer in individuals who were treated with Remicade compared with controls. This is a new drug. Of course, long term safety studies are ongoing.
What does Remicade cost?
This novel medication is very expensive. Of course, there is no generic substitute. The usual dose is 5 mg per kilogram of weight adminstered over a two hour infusion. The average 70 kilogram person (154 pounds) would require 350 milligrams. The cost of this infusion would be about $2500. When given for proper indications, most insurance carriers will cover all or part of the cost. This varies by insurance company, so preauthorization is usually required.
Mount Sinai-National Jewish Web site: http://nationaljewish.mountsinai.org/
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