Friday, February 7, 2014

Connective Tissue Disease - Associated Interstitial Lung Disease

I am looking forward to Tuesday's Interstitial Lung Disease Support Group at my university hospital. It will feature a doctor who will present the latest research and treatment options regarding connective tissue diseases associated with ILD. We have several people in our group struggling with an ILD as well as rheumatoid arthritis or Scleroderma. There is a medical connection of these two diseases groups. I will take notes and report back. Below is the handout that members of the group have been given so we have some background information before the meeting.


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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