Medications by Noah Greenspan

PT, DPT, CCS, EMT-B and Robert J. Kaner, MD

“Drugs don’t work if patients don’t take them (properly).” – Former US Surgeon General, C. Everett Koop, MD

The medical treatment for ILD’s can be as diverse and complex as the conditions themselves. This is yet another reason why it behooves you to seek out a physician who truly specializes in the science and art of caring for patients with ILD’s and a COE.

One crucial point is that for most ILD’s there are no cures and, in some cases, few if any treatments will have a direct impact on the progression of the disease or the lungs themselves. In conditions in which the inflammatory trigger is known, it is imperative that the offending agent be removed completely and if it cannot, may require removing yourself from the environment. In still other causes of ILD, the treatment is targeted toward the underlying cause or condition, thereby hoping to improve the respiratory component of the disease and its associated symptoms. 

In all these cases, outcome and prognosis with respect to disease severity and life expectancy is highly dependent upon early and correct diagnosis, and specific targeted treatment. For that reason, what you can, could, or should do is way beyond the scope of this book and its authors. Rather, we will give a basic and brief description of some of the medications available to treat ILD’s with the super caveat that every treatment regimen must be expertly tailored to the individual patient, based upon their individual condition, and monitored and modified by their own ILD specialists. 

NOTE: As you read this chapter, please keep in mind that like any medication, there are often many potential side effects. Therefore, any decisions about potential risks and benefits must be decided with your own physician(s).

Corticosteroids (Prednisone) 

Corticosteroids, also known simply as “steroids” are anti-inflammatories that can reduce symptoms by suppressing the activity of the immune system, decreasing inflammation and edema (swelling) in the airways, lungs, and other body parts. 

Mycophenolate mofetil/mycophenolic acid (Brand Names: Cellcept and Myfortic)

Mycophenolate is an immunosuppressant that has anti-inflammatory and anti-fibrotic properties. It can reduce symptoms by suppressing the activity of the immune system. In some cases, this medication can help reduce the need for corticosteroids.

Azathioprine (Brand Name: Imuran)

Azathioprine is an immunosuppressant that reduces symptoms by suppressing the activity of the immune system to prevent the body’s immune system from attacking its own cells, that can lead to pulmonary fibrosis. It is often used to treat pulmonary fibrosis and other conditions that cause pulmonary fibrosis, like Rheumatoid Arthritis. In some cases, this medication can help reduce the need for corticosteroids and is often used when other medications are poorly tolerated due to side effects. 

Cyclophosphamide (Brand Name: Cytoxan)

Cyclophosphamide is a chemotherapeutic/anti-inflammatory agent that can reduce symptoms by suppressing the activity of the immune system to prevent the body’s immune system from attacking its own cells, by destroying specific inflammatory cells in the body. In some cases, this medication can help reduce the need for corticosteroids, or can be used in combination with corticosteroids or in cases when corticosteroids are contraindicated. 

Pirfenidone (Brand Names: Esbriet, Pirfenex, Pirespa)

Although the exact mechanism of action of Pirfenidone is not completely understood, it seems to have anti-inflammatory and anti-fibrotic effects. Pirfenidone does not reverse fibrosis. However, it may slow the progression of fibrosis in some patients with PF/IPF. This is another argument for early diagnosis and treatment.

Nintedanib (Brand Name: Ofev)

Nintedanib has anti-inflammatory and anti-fibrotic effects. It acts on the pathways that cause scarring in the lungs in some patients with PF/IPF, and scleroderma associated ILD. Nintedanib does not reverse fibrosis. However, it may slow the progression of fibrosis in some patients with mild to moderate PF/IPF. 

 

Tocilizumab (Brand Name: Actemra)

Tocilizumab is a biologic medication that has anti-inflammatory effects. It may slow the rate of decline of pulmonary function in some patients with PF/IPF, and scleroderma associated ILD.

Once again, the medical treatment for ILD’s can be as diverse and complex as the conditions themselves. This is yet another reason why it behooves you to seek out a physician who truly specializes in the science and art of caring for patients with ILD’s and a COE.

Rituximab (Brand Name: Rituxan)

Rituximab may offer a safe and effective therapeutic intervention in a subgroup of patients with severe, progressive ILD unresponsive to conventional immunosuppressive medications. It is the go-to for people failing standard therapy for scleroderma, myositis, and other Connective Tissue Disorders (CTD’s).

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