What Is Connective Tissue Disease associated (ILD)?

Connective tissue diseases (CTDs) consist of a spectrum of diseases that affect multiple parts of the body including your joints, skin, eyes, gastrointestinal track, heart, and lungs. These conditions include rheumatoid arthritis, scleroderma, systemic lupus erythematosus, Sjögren’s syndrome, polymyositis/dermatomyositis, and mixed connective tissue disease.

Each of the CTDs impacts the lungs in different ways. CTDs can cause serious complications for your pulmonary system, including bleeding in your lungs, inflammation and scarring of the spaces between the lung's air sacks and the blood vessels. This kind of inflammation and scarring are typical of interstitial lung disease (ILD), and when CTDs cause ILD (CTD-ILD), it can prevent oxygen from being absorbed in the lung and can lead to shortness of breath, cough, and fatigue.

What are the Symptoms of CTD/ILD?

While issues with your lungs can sometimes be the only symptoms that you experience with an underlying CTD, most patients with CTD experience symptoms across multiple organ systems. Symptoms include:

  • Shortness of breath, painful breathing, or difficulty breathing
  • Chronic cough
  • Weakness and fatigue
  • Nosebleeds
  • Bloody phlegm
  • Rashes
  • Mouth ulcers
  • Anemia
  • Difficulty concentrating

Diagnosis of Connective Tissue Disease associated (ILD)

An accurate diagnosis requires a careful medical history, a thorough physical exam, blood work and an assessment for other non-pulmonary manifestations of a CTD, including a CAT scan to assess the pattern of lung scarring to determine if you have CTD-ILD. If your pulmonologist is concerned about an underlying CTD, you may be referred to a rheumatologist for additional testing in order to identify which specific CTD you have as it will affect treatment and disease trajectory.

CTD-associated ILD is typically a progressive condition and the two diseases may progress independent of or concurrent with each other. The trajectory of each CTD is very different, making it very important to diagnose your specific CTD. Pulmonologists at Penn work very closely with the rheumatologists to diagnose and subsequently provide thoughtful, comprehensive treatment for your CTD-ILD.

Treatment at Penn

Because the degree of inflammation and fibrosis seen in CTD-ILD varies by CTD type and by individual, your medical team will create a treatment plan specified to your individual needs. Some patients may require oxygen therapy, pulmonary rehabilitation exercise programs, symptom therapy, and, in advanced cases, lung transplantation.

Typical treatments for CTD-ILD patients include drugs to slow the progression of your disease, such as corticosteroids and immunotherapies.

Some of the immunotherapy medications can also be used to treat other symptoms of CTDs such as joint and skin symptoms. You should be aware that sometimes the lung symptoms will continue to progress even though the other organ systems (like joint and skin) seem well controlled. All of these drugs have potential side effects and require ongoing monitoring, making Penn's interdisciplinary team approach between pulmonologists and rheumatologists ideal for managing your condition.

If your CTD-ILD has caused enough damage that surgery is required, know that Penn is a national leader in lung transplantation. Penn Medicine's specialized surgeons offer medical treatments not available at other centers and perform four times as many lung transplants as any other hospital in the region. Penn specialists excel in procedures in the area of chest, lung and artificial breathing operations, outperforming any other area hospital.

When you choose Penn, you choose to work with a dedicated team offering the most innovative treatments and surgeries. Penn physicians continue to lead the field, advancing the science of lung care.

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