Interstitial lung disease (ILD) covers a broad category of lung diseases characterize
by extensive scarring (fibrosis) of the lungs. In most cases the cause of this scarring
remains unknown. Some of the known causes include occupational and environmental
exposures (asbestos, silica, animal proteins, and metal dust). They may also be
a consequence of medical treatment with drugs or radiation. They may be associated
with rheumatologic and connective tissue diseases (systemic lupus erythematosus,
rheumatoid arthritis, scleroderma, polymyositis/dermatomyositis).
The NS-LIJ ILD Program is designed to the help in the diagnosis and care of patients
with this group of disorders. After a complete evaluation, our physicians will discuss
the diagnosis and possible management of your disease. Treatment may include standard
immunosuppressive therapy, newer antifibrotic (anti-scarring, therapy or even consideration
for lung transplantation.
Pulmonary Hypertension is high blood pressure (hypertension) in the lungs arteries
(pulmonary arteries) that supply all the oxygen–poor blood returning from all the
other organs to the lungs. There are a number of reasons this condition may result.
For example, the blood vessels of the lung (pulmonary arterioles) may have narrowed,
thus restricting blood flow through the lungs. Alternatively, the condition may
be caused by heart disease of the left side of the heart, which causes blood to
back up into the lungs, raising the blood volume and pressure in the pulmonary vessels.
Pulmonary hypertension is diagnosed by measuring the blood pressure in the pulmonary
arteries.
Primary pulmonary hypertension (PPH) occurs with no known cause, whereas secondary
pulmonary hypertension is due to another, diagnosed disease. PPH is rare, (500 to
1,000 new cases per year) and can occur in men, women and children of all ages.
It is most common in women between 20 and 40 years old. PPH is rare in children
and usually associated with a congenital heart defect. Secondary pulmonary hypertension
(SPH) is more common and occurs with a wide variety of lung and heart disease although
the exact causes are unknown. Some of the causes of secondary pulmonary hypertension
(SPH) include:
- HIV
- Raynaud's syndrome
- Sickle cell anemia
- Lung disease (e.g., pulmonary fibrosis, COPD & emphysema)
- Heart problem (e.g., congenital heart disease or mitral valve stenosis)
- Pulmonary embolism (blood clot(s) usually from the legs that break
up and become lodged in a blood vessel in the lung).
The ability to cure pulmonary hypertension is related to the cause of the disease.
There is no cure yet for primary hypertension, but in recent years, physicians have
developed a number of approaches that can successfully manage the condition and
improve the patient’s quality of life. Secondary pulmonary hypertension may sometimes
be cured by treatment of the underlying condition. In either case, it is important
to identify the condition as quickly as possible and seek out qualified treatment
before permanent damage results to the lungs or the right side of the heart.
People with interstitial lung disease usually develop symptoms when they develop
worsening pulmonary hypertension. Usually, there are no symptoms in the early stages
of pulmonary hypertension. The symptoms that eventually appear can vary widely from
patient to patient. They may include:
- Fatigue or tiredness
- dizziness
- Fainting (syncope)
- shortness of breath
- Swelling (edema) in the ankles or feet
- Bluish skin or lips (cyanosis)
- chest pain/discomfort
- Coughing
- Distended neck veins
- Enlarged liver
- Swollen abdomen
It easy usually suspected by a physician when the patients complains of problems
mentioned above. A history of the patients and physical examination usually points
in the direction of the diagnosis and test that must be ordered to confirm the diagnosis.
- Chest x-ray. This imaging test offers the physician a picture of
the general size, shape and structure of the heart and lungs. One of the things
the physician will check is whether the right side of the heart is enlarged.
- CAT scan of lung usually with dye (contrast). This imaging offers
a much more detailed picture of the lungs, heart, and blood vessels. This test often
in conjunction with the history and physical examination can identify a specific
cause of interstitial lung disease and pulmonary hypertension.
- Electrocardiogram (EKG). EKG is a test that measures the heart’s
electrical activity. It is designed to detect any abnormal rhythms (arrhythmias),
which may be signs that the heart or parts of the heart are overly stressed.
- Echocardiogram. Echocardiogram of the heart and major arteries
is a noninvasive test that uses sound waves to track the structure and function
of the heart. A moving image of the patient’s beating heart is shown on a monitor,
where a physician can study the heart’s thickness, size and function.
- Pulmonary function tests (PFTs). PFTs are painless and noninvasive
test in which the patient blows into a special device that measures and evaluates
how well the lungs are functioning. This may include and arterial blood gas study
(ABG) where blood is taken from an artery and analyzed for the amount of gas is
present especially oxygen.
- Perfusion lung scan. A noninvasive test used to show the pattern
of blood flow in the lung. The test involves the injection of a radiopharmaceutical
(very small amount of radioactive material) into an arm vein before scanning the
lung with a special (gamma) camera. Pictures are then developed which gave a very
good idea of lung flow in the lung and is especially useful in diagnosing blood
clots a cause of secondary pulmonary hypertension.
- Right–heart cardiac catheterization. A thin plastic tube (catheter)
is passed through a vein into the right side of the heart and then into the pulmonary
artery where it is used to obtain a precise measure of blood pressure and flow in
the lung. The physician will also measure and evaluate the pumping ability of the
right side of the heart. The ability of the lung arteries to widen in response to
specific medications will also frequently be measured. This will guide the physician
as to what medications may be useful to treat the pulmonary hypertension. The procedure
it usually performed in a specialized catheterization laboratory.
- Pulmonary angiogram. A test used to measure circulation in the
lungs and to visualize clots in the lung on an x-ray machine (fluoroscopy). It is
now uncommonly used in special circumstances and has been largely replaced by a
noninvasive CAT scan study with dye. Pulmonary angiogram involves the insertion
of a thin catheter into the pulmonary artery through which an iodine dye is injected.
The physician can then see an image of any blood clots present in the lung, and
track the circulation of blood through the lung's blood vessels.
- Fiberoptic bronchoscopy. Fiberoptic bronchoscopy is a technique
where a thin fiberoptic flexible tube is passed through a nostril or mouth of the
patients who has received topical anesthetic (spray or jelly) and sedation. It is
guided passed the voice box into the wind pipe and airways and allows specimens
tube taken from the lung. It may be used in establishing the diagnosis of an interstitial
lung disease. Flexible bronchoscopy is usually performed as and ambulatory procedure
and does not require hospitalization. Diagnostic material is taken by several techniques.
These include bronchoalveolar lavage and (BAL) where small amounts of saline are
use to wash bronchial tubes and the lung tissue beyond. Actual biopsies of the bronchial
tubes or lung can be obtained by small brushes and forceps devices.
- Video Assisted Lung and Chest Surgery (VATS). Surgery is sometimes
required to establish the diagnosis of interstitial lung disease. In our center
this is almost always by a minimally invasive surgical technique known as video
assisted thoracic surgery (VATS). Although patients requiring VATS are admitted
to the hospital most do not need intensive care and usually leave in 1 to 3 days.
In many cases they may also be back to normal activities within a week. Surgeons
operate through 2 to 4 tiny openings (less than an inch long) between the ribs while
viewing the patient's lungs on a television monitor. Special instruments allow the
lung tube safely biopsied.
- Location: 410 Lakeville Rd., 1st Floor, Suite 107
- Clinical Director: Arunabh Talwar, MD
- Nurse Coordinator: Sophie Dedopoulos, NP
- Appointment Contact: (516) 465-5400