Patient Information:
Connective tissue disease-associated
interstitial lung disease (CT-ILD)
Introduction
Connective tissue disease-associated interstitial lung disease (CT-ILD)
is a type of lung disease that occurs in a small number of patients with a connective
tissue disease diagnosis. Examples
of connective tissue diseases (also known as rheumatologic , collagen vascular,
or autoimmune diseases) include: scleroderma, rheumatoid arthritis, Sjogren’s
syndrome, systemic lupus erythematosus, polymyositis, dermatomyositis, and
mixed connective tissue disease. In many cases, patients are diagnosed with the
connective tissue disease first and develop CT-ILD later, although in some
cases, the opposite is true.
CT-ILD causes inflammation and/or scarring (fibrosis) of the lungs. The
exact mechanism that leads to lung damage is unknown.
Some patients with CT-ILD are asymptomatic. Common symptoms and signs
of CT-ILD include:
· Shortness
of breath with activity
· Cough
· Fatigue
· “Crackles"
on examination of the chest with a stethoscope
· Symptoms
and signs of a connective tissue disease (for example, joint pain/swelling,
rash, dry eyes, dry mouth, acid reflux)
Diagnosis
The diagnosis of CT-ILD requires input from pulmonologists,
radiologists, and in some cases, pathologists experienced in the evaluation of
patients with interstitial lung disease (diseases of the lung tissue that often
cause inflammation or scarring). In many cases, a face-to-face discussion among
these different physicians is necessary for an accurate diagnosis.
In making a diagnosis of CT-ILD, your doctor will start by conducting a
thorough history and physical examination. This will include discussing your
medical history, reviewing medications and potential occupational and
environmental exposures, listening to your chest with a stethoscope to check
for extra sounds (crackles), and carefully examining your skin and joints.
In addition to a careful medical history and physical examination, the
following tests may be conducted by your physician to help make an accurate
diagnosis.
· Pulmonary Function Testing (PFT) – This test involves a series of breathing maneuvers
that measure the lungs’ airflows, the volume of air in your lungs, and the ability
of your lungs to extract oxygen from the air. This allows your doctor to
objectively assess the function of your lungs.
High Resolution Computed Tomography (HRCT) – This is a special type of CT (aka “CAT”) scan that provides your
doctor with high-resolution images of your lungs. Images should be taken in several
different ways: lying on your back (supine), lying on your chest (prone), and
with you breathing air out of your chest ·
(dynamic expiration). These various images are
extremely valuable in determining whether or not you have CT-ILD. Having a HRCT
is no different than having a regular CT scan but requires the radiologists to
follow a special protocol. A HRCT is performed on an open-air table and takes
only a few minutes.
·
Serological Tests –
Blood tests (serologies) for the presence of various antibodies may be ordered
to evaluate you for a connective tissue disease. Some patients with connective
tissue diseases develop lung problems before the more typical features are
apparent; in these cases, serologies may be the only evidence of the diagnosis
of CT-ILD.
·
Referral to a rheumatologist: For some patients, interstitial lung disease is
the first manifestation of a connective tissue disease. If this is the case,
you may be referred to a rheumatologist for further evaluation.
Treatment
There have been a few recent clinical trials evaluating
the efficacy of medications for CT-ILD. Therapeutic options focus on the use of
anti-inflammatory or immunosuppressive medications. You may recognize some or all of these medications as they
may have been prescribed to you for your connective tissue disease in the past.
The following are the most common anti-inflammatory/immunosuppressive medications
used to treat CT-ILD:
·
Corticosteroid (prednisone)
·
Cyclophosphamide (Cytoxan)
·
Mycophenolate mofetil (Cellcept)
·
Azathioprine (Imuran)
If you have
questions about the above medications, we have educational handouts for each
one.
Pulmonary rehabilitation is an important and effective
treatment for patients with CT-ILD. Pulmonary rehabilitation is a structured
exercise and educational program designed for patients with chronic lung
disease. Careful attention to common associated medical problems such as gastroesophageal reflux
disease and pulmonary hypertension may also be important. Lastly, lung
transplantation may be an effective treatment option for selected patients who have
failed medical therapy and have progressive symptoms.
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