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October 2009

Early Treatment of ILD Improving Survival

Harold R. Collard, M.D.
Pulmonologist
Director of the UCSF Interstitial Lung Disease Program

Interstitial lung disease (ILD) is a group of acute and chronic lung disorders with variable degrees of pulmonary inflammation and fibrosis. Many practitioners have a fatalistic view about ILD, believing that a specific diagnosis and prompt management is of limited importance since prognosis and treatment response is universally poor. As a result, many patients receive a delayed diagnosis and are not referred to appropriate specialists.

New drugs, however, are being tested that may halt or slow the progression of fibrosis. In addition, lung transplant is an effective option in certain cases. A proactive approach to the diagnosis and management of patients with ILD and comprehensive medical care can result in improved quality of life and survival.

There are many specific forms of ILD with different prognosis and management. The most well-known is idiopathic pulmonary fibrosis, or IPF. Other forms of ILD include hypersensitivity pneumonitis, sarcoidosis and connective-tissue-associated ILD, or rheumatological disease.

Diagnosis

A prompt and accurate diagnosis is critical to the management of patients. ILD should be suspected in patients with chronic, unexplained shortness of breath or cough, crackles on chest auscultation, or abnormal interstitial marking on chest radiograph. These patients should be promptly referred to a pulmonologist or ILD Center for further evaluation.

The diagnosis and management of ILD is complex and requires the expertise of pulmonologists, radiologists and pathologists. Prognosis, identification to extrapulmonary manifestations and comorbidities, choice of medication and consideration for transplant all depend on an accurate diagnosis and staging of the disease.

High-resolution Computed Tomography (HRCT)

All patients with suspected or confirmed ILD should have an HRCT with supine, prone and expiratory images, even if their chest radiographs are negative. HRCT can accurately diagnosis most forms of ILD and in many cases obviates the need for surgical lung biopsy.

Treatment

Treatment depends on the specific form of ILD. Many forms are characterized by significant inflammation. These include connective tissue-associated ILD (e.g. scleroderma, rheumatoid arthritis), sarcoidosis and hypersensitivity pneumonitis. Typical therapies for these conditions are listed below. IPF appears to be different. It may represent a primary disorder of fibroproliferation, or scar formation.

Research has shown that early treatment can significantly prolong the survival of patients with inflammatory forms of ILD by halting or slowing the progression of fibrosis. The following drugs are felt to be effective in treating patients with inflammatory forms of ILD:

  • Azathioprine
  • Cyclophosphamide
  • Methotrexate
  • Mycophenolate
  • Prednisone

The choice of agent depends on many factors including the specific diagnosis.

Treatment of IPF is controversial, but there are several promising, new therapies. High-dose acetylcysteine and pirfenidone, a novel antifibrotic agent, have both been associated with slowed progression of disease in large, multicenter trials of patients with IPF.

Other therapies including bosentan, ambrisentan, sildenafil and Coumadin are under investigation, based on suggestive preliminary data.

Lung Transplantation

An increasing number of patients with ILD undergo lung transplants. The disease is now the leading indication for lung transplantation at most centers in the United States. Lung transplantation is a particularly effective treatment for patients under 65 years old without significant comorbidities.

Some transplant centers, such as UCSF Medical Center, perform lung transplants on older patients with ILD. Considering medical and emotional preparations for transplant and the unpredictability of many forms of ILD, early referral for lung transplantation evaluation is recommended.

UCSF ILD Program

The UCSF Interstitial Lung Disease Program specializes in the comprehensive care of people with all forms of ILD. We provide state-of-the-art diagnosis and treatment, working as a team to review each case thoroughly and optimize disease and symptom management. We communicate directly with patients and their referring doctors to assure continuity of care and appropriate follow-up.

We hold seminars on ILD for patients and their families and offer a monthly support group, called Living Well with ILD. We also offer continuing medical education sessions on ILD, as well as a quarterly newsletter.

Our program conducts both basic and clinical research. We are a member of the National Institute of Health (NIH)-sponsored Idiopathic Pulmonary Fibrosis Clinical Research Network and are involved in most major clinical trials for patients with ILD. Patients have the opportunity to participate in these research trials if they wish.

For more information, visit our Web site at www.ucsfhealth.org/ild or contact us at (415) 353-2577.

Other Resources

For more information, contact the Physician Referral Service at UCSF Medical Center:

Phone (888) 689-UCSF or (888) 689-8273
Email referral.center@ucsfmedctr.org