Overview

Overview

The Interstitial Lung Disease Program provides extensive diagnostic testing and treatment options for patients with:

Often our physicians are able to diagnosis and manage our patients’ conditions with less invasive treatments, minimizing the use of steroids.

Respiratory Institute pulmonologists also work closely with the Department of Rheumatic and Immunologic Diseases to treat connective tissue diseases, including:

We bring a multidisciplinary approach to the diagnosis and therapy of interstitial lung diseases, including weekly multi-disciplinary case review by pulmonologists, rheumatologists, thoracic radiologists and pulmonary pathologists with extensive experience with ILD.

For diagnosis, our bronchoscopy team and thoracic surgeons are able to perform both standard and advanced procedures in cases where biopsy is necessary. However, thanks to our extensive experience, invasive diagnostic procedures are often unnecessary.

Pulmonary rehabilitation is available at Cleveland Clinic’s main campus as well as at several of our community hospitals.

For oxygen therapy needs, we offer pulmonary function testing and assessment. For patients with high oxygen requirements, we provide transtracheal oxygen catheter placement.

We also have access to a wide range of clinical trials as well as non-steroid therapies for ILD.

For patients with advanced lung diseases, we work closely with the lung transplant team, offering an integrated evaluation.

For more information or to make an appointment, please contact our ILD Care Coordinator at 216.445.4538.

Pulmonary Fibrosis Support Group

This meeting is held for patients, caregivers, family and friends of those diagnosed with pulmonary fibrosis. Most meetings involve a brief presentation by an expert on some aspect of dealing with pulmonary fibrosis, such as understanding the disease, available treatments, and the basics of supplemental oxygen.

The presentation is followed by a question/answer session and then a discussion led by the group.

If you would like to participate or have additional questions, please contact pfsupport@ccf.org.

2017 dates

  • Tuesday, June 20 from 6:30 p.m. - 8:30 p.m.
  • Tuesday, September 19 from 6 p.m. - 8 p.m.
  • Tuesday, December 19 from 6 p.m. - 8 p.m.

Location
Cleveland Clinic Independence Family Health Center
5001 Rockside Rd, Crown Center II
Independence, OH 44131

Health Information

Health Information

Interstitial lung diseases (ILDs) is a group of conditions that cause scarring to the lungs. ILDs include forms of pulmonary fibrosis and interstitial pneumonia as well as ILDs associated with connective tissue diseases, tobacco use, and exposure to environmental and occupational toxins.

Free Treatment Guide: Learn about unique ILDs treatment options.

Learn common symptoms and causes as well as ways we diagnose and treatment the following types of interstitial lung diseases:

Idiopathic Interstitial Pneumonia

Pulmonary Manifestations of Connective Tissue Diseases

ILDs Related to Tobacco Use

  • Respiratory Bronchiolitis-associated ILD
  • Desquamative Interstitial Pneumonia
  • Langerhan's Cell Histiocytosis

Other Interstitial Lung Diseases

Doctors

Doctors

Research & Clinical Trials

Research & Clinical Trials

A double blind, randomized, placebo-controlled trial evaluating the efficacy and safety of nintedanib over 52 weeks in patients with progressive fibrosing interstitial lung disease (PF-ILD)

Sponsored by Boehringer Ingelheim Pharmaceuticals, this is a Phase III trial investigating the efficacy and safety of twice daily 150 mg Nintedanib (versus placebo) in patients with progesttive fibrosing interstitial lung disease (PF-ILD). The primary endpoint is the annual rate of decline in forced vital capacity (FVC) in mL over 52 weeks.

Eligibility: Patients aged ≥ 18 years with PF-ILD, defined as patients who present with featured of diffuse fibrosing lung disease of ≥ 10% extent on HRCT and whose lung function and respiratory symptoms or chest imaging have worsened despite treatment with unapproved medications used in clinical practice to treat ILD. FVC ≥ 40% predicted; DLCO 30% - 80% predicted (corrected for Hb). Current use of immunosuppressants is not permitted.

PRINCIPAL INVESTIGATOR:
Leslie Tolle, MD

STUDY COORDINATOR:
Ron Wehrmann, RRT | 216.445.0574


A Double-Blind, Randomized, Placebo-controlled Trial Evaluating Efficacy and Safety of Oral nintedanib Treatment for at least 52 Weeks in Patients with “Systemic Sclerosis Associated Interstitial Lung Disease” (SSc-ILD)

Sponsored by Boehringer Ingelheim Pharmaceuticals, this is a Phase III trial investigating the efficacy and safety of twice-daily 150 mg nintedanib (vs. placebo) in patients with systemic sclerosis associated interstitial lung disease. The primary endpoint is the annual rate of decline in forced vital capacity (FVC) in mL over 52 weeks. ELIGIBILITY: Patients ≥ 18 years diagnosed with systemic sclerosis associated interstitial lung disease based on classification according to the most recent ACR/EULAR criteria and HRCT (within previous 12 months); onset of systemic sclerosis (defined as first non-Raynaud symptom) not more than 5 years ago; extent of fibrotic disease in the lung ≥ 10%; FVC ≥ 40% of predicted; DLCO 30% to 89% of predicted (corrected for Hb).

PRINCIPAL INVESTIGATOR:
Kristin Highland, MD, MS

STUDY COORDINATOR:
Ron Wehrmann, RRT | 216.445.0574


Genetic Risk for Granulomatous Interstitial Lung Disease

The objective of this NIH-supported study is to identify genetic risk factors for sarcoidosis. 

ELIGIBILITY:  Patient must be Caucasian.  Biopsy proven sarcoidosis or Lofgren’s syndrome and at least one of the following, documenting pulmonary disease involvement due to sarcoidosis: 1. A biopsy from the lung (either via bronchoscopy or otherwise) or chest lymph nodes demonstrating granulomas and or (2) Pulmonary parenchymal involvement with Scadding chest x-ray stage I, II, III, IV, in the past or present and/or (3) Abnormal spirometry and/or DLCO (<80% predicted).  Exclusion Criteria: Positive lung washing or biopsy cultures for fungi or mycobacterial disease.  Patient’s inability to undergo venipuncture.

PRINCIPAL INVESTIGATOR:
Daniel Culver, DO

STUDY COORDINATOR:
Christopher Estling | 216.445.8951


IP-PRO Registry

Sponsored by Boehringer Ingelheim, this is a registry with the overall goal to collect data and biological samples that will support future research studies, as well as obtain for future research studies, biological samples as serial time points that are matched to the well characterized IPF participants enrolled in this registry. This registry is looking for patients who are > 40 years old and have established a new diagnosis of IPF by the enrolling subspecialty center (as defined by ATS/ERS/JRS/ALAT criteria). 

PRINCIPAL INVESTIGATOR:
Daniel Culver, DO

STUDY COORDINATOR:
Ron Wehrmann, RRT | 216.445.0574

For Medical Professionals

For Medical Professionals

Patient Referrals

Our secure online service, Dr.Connect, provides referring physicians access to patient’s treatment progress with streamlined communication from Cleveland Clinic physicians to your office, allowing continued participation in the ongoing care of patients. With the best possible treatment plans and coordinated care, our team approach benefits both the patient and the referring physician.

Education

Pulmonary and Critical Care Medicine Fellowships

Our pulmonary and critical care medicine fellowships provide board-certified general internists with the tools necessary to care for patients, who have complicated lung diseases and critical illnesses. During the three-year training period, which includes an 18-month core program and 18-month subspecialty track, fellows are exposed to a wide variety of medical problems in both the inpatient and outpatient settings.

CMEs

Cleveland Clinic's Respiratory Institute offers continuing medical education courses through the Center for Continuing Education. We are providers of AMA-approved continuing medical education (CME) units for physicians and physician assistants, and of continuing education units by the Ohio Nurses Association for nurses and by the Ohio Respiratory Care Board for respiratory therapists.

Cleveland Clinic publishes an online medical reference, the Disease Management Project, which provides nationally-established treatment guidelines for the most common diseases and conditions.

Glossary of Terms

Glossary of Terms

Azathioprine

An immunosuppressant used for almost all ILD that can be treated with immunosuppressants, except for IPF, PAP, LAM and smoking-related ILD (RBILD, DIP and PLCH). It works by suppressing proliferation of immune cells and is taken daily, usually split into morning and evening doses. Typical doses range from 75 to 200 mg total per day. The most common side effects include abdominal cramping, diarrhea, hair loss and fatigue. Toxicities include low blood counts and liver inflammation, although the liver inflammation is less prominent than with methotrexate and leflunomide. Its toxicity is generally monitored with blood work every four to 12 weeks.


Bronchoscopy

A procedure to examine the airways leading to your lungs, which is usually performed on an outpatient basis (with no hospital stay). It involves passing a narrow, flexible instrument through the mouth or nose and into the airways and is helpful for diagnosing certain types of interstitial lung disease, most commonly including sarcoidosis, chronic beryllium disease, lymphangioleiomyomatosis, pulmonary alveolar proteinosis, and pulmonary Langerhans cell histiocytosis. It also is useful as a research tool, to exclude infections. During a bronchoscopy, additional procedures may be performed including bronchoalveolar lavage, bronchoscopic biopsies (taking a sample of airways mucosa or of alveoli), and needle aspiration sampling of lymph nodes or masses.


Bronchoalveolar lavage (BAL)

A procedure performed during bronchoscopy that involves washing out a segment of the lung with salt water to remove cells and proteins from the air sacs (alveoli). It is most frequently used for research sampling of the alveolar cells, checking for infection and looking at the alveoli cell components. The proportions of types of immune cells in the BAL can suggest or rule out some types of ILD. Except for rare situations, BAL does not give a definitive diagnosis of any ILD.


Chest X-ray

Also known as a chest radiograph, X-rays are useful for follow-up of ILD, and in certain cases is sufficient for diagnosis, such as for sarcoidosis. However, today, most patients also have a diagnostic chest CT. The chest X-ray is preferred as a tool for helping monitor the progress of the disease, in conjunction with breathing tests and symptoms


Cyclophosphamide

An alkylating agent (one that changes the metabolism), slowing or stopping cell growth. For this reason, cyclophosphamide is used mostly for the treatment of cancer. Since the drug also decreases the immune system’s response to various diseases, it is used to treat other medical conditions, such as interstitial lung disease – especially in cases of scleroderma associated with lung compromise.

Can be administered either orally (by mouth) or intravenously (by IV). Side effects are numerous and include an increased long-term risk of developing bladder cancer. For this reason, cyclophosphamide is used only for short periods of time (six months to one year). Other adverse drug reactions are nausea, vomiting, stomachache, diarrhea, hair loss, increased risk to infections and hemorrhagic cystitis.

With this medication, close follow-up is needed, with monthly blood work. It should be taken early in the morning with an adequate fluid intake.


Cyclosporin

An immunosuppressant drug, one of the first used in organ transplantation and remains widely used today. It reduces the activity of the immune system by interfering with the activity and growth of T cells (a type of blood cell that fights infection).

Cyclosporin also is used to treat other different diseases, including Interstitial Lung Disease, especially these related with connective tissue diseases. It may be administered either orally (by mouth) or intravenously (by IV). Side effects may include kidney toxicity, gingival hyperplasia, peptic ulcers, fever and diarrhea. Today, this medication is being used less frequently, given newer, more potent and less toxic options.


Diffusing capacity of the lungs for carbon monoxide (DLCO)

This is a test of gas exchange at rest. It measures the ability of the lungs to transfer oxygen out of the atmosphere and into the bloodstream. DLCO is typically impaired in ILD due to thickening of the space between the air sacs and the blood compartment (capillaries) from inflammation or scarring.

DLCO is usually followed over time to look for progression of the disease, but it is more variable from day-to-day than the spirometry and also more affected by varying techniques in different pulmonary function labs. Therefore, a change of at least 10 to 20 percent is necessary to confidently suspect that there is an actual difference between tests. Some causes of low DLCO besides infiltration in the wall of the air sac include pulmonary hypertension, low hemoglobin levels (anemia) and technical difficulties with the test.


High resolution chest CT

A CT scan of the lungs using specific protocols. This type of CT scan is best used at the time of diagnosis and later only when an unusual aspect of the disease occurs which requires the physician to look for complications or additional diseases.

For ILD, this type of chest CT should be obtained without IV contrast (dye), and with thin sections to allow for detailed examination of the lung tissue. With an experience CT reader, many ILDs can be diagnosed with a high degree of confidence using CT alone, without the need for a biopsy. For example, IPF can be diagnosed with over 90 percent confidence when the CT scan demonstrates certain characteristic findings. In those cases, the risk of a surgical lung biopsy usually outweighs the small chance of finding a different diagnosis than IPF.

FVC is commonly reduced when the lungs are stiffened by scar or inflammation, the breathing muscles are weak or different techniques are used to obtain it. A significant change of FVC over time (typically 5 to 10 percent) indicates a clinically useful improvement or an important deterioration. The forced expiratory volume in 1 second (FEV1), the amount exhaled in the first second of spirometry, may be disproportionately low when there is obstruction of airflow. There are several standard equations to predict normal values, so it is important to look at raw numbers, not percent predicted values, when comparing tests from two different institutions.


Leflunomide

An immunosuppressant used mainly for sarcoidosis and hypersensitivity pneumonitis, though it may theoretically be useful for other ILD with predominant lymphocyte involvement. It works by suppressing proliferation of a type of immune cells, lymphocytes. Leflunomide is taken once daily, with the most common dose being 20 mg daily. The most common side effects include abdominal cramping, diarrhea and hair loss. Toxicities include liver inflammation, lung inflammation and neuropathy (burning, tingling or numbness usually starting in the toes and fingers). Its toxicity is monitored with blood work, initially every four weeks for the first six months, then less frequently. Because it remains in the system for months after stopping it, cholestyramine (a cholesterol medication) must be taken for two days to remove it via the stool if an individual needs to stop leflunomide quickly.


Lung volumes (plethysmography or helium dilution studies)

These pulmonary function tests measure the total gas volume in the lungs as well as the amount of gas remaining in the lungs after normal and forced expiration. The purpose of measuring these is to look for trapped air remaining in the lungs after expiration, confirm the presence of low forced vital capacity, and measure the relative contributions of lung tissue, chest wall stiffness and airways obstruction to low spirometry values.


Methotrexate

An immune suppressant that is typically taken once weekly. It is used most commonly for sarcoidosis, and sometimes for ILD associated with rheumatologic conditions. Typical doses range from 7.5 to 20 mg weekly. The most common side effects include nausea, fatigue, hair loss and headache. Methotrexate can cause liver enzyme elevations (and occasionally liver fibrosis), low blood counts and sometimes lung inflammation. Its toxicity is generally monitored with blood work every four to 12 weeks.


Mycophenolate

An immunosuppressant that works by suppressing proliferation of immune cells. It is newer and more expensive than some other immune suppressants. It is used for almost all ILD that can be treated with immunosuppressants—notable exceptions include IPF, PAP, LAM, and smoking-related ILD (RBILD, DIP and PLCH).

Mycophenolate is taken daily, usually split into morning and evening doses, with doses typically ranging from 1000 to 3000 mg total per day. The most common side effects include abdominal cramping, diarrhea and fatigue. Toxicities include low blood counts. Its toxicity is generally monitored with blood work every four to 12 weeks.


Oxygen therapy

Normally, the oxygen level (saturation) in the blood does not drop with exercise. However, in ILD, the lungs often cannot keep up with the increased blood flow that occurs during exercise, so oxygen saturation may drop during exertion. If the saturation is too low, it can lead to consequences such as breathlessness, or low oxygen delivery to important organs like the brain or the heart.

While this desaturation may be tolerable in mild forms or for brief periods, over time it can lead to excess strain on the right side of the heart. The effects on the brain are likewise thought to be unfavorable, based on studies of mountain climbers. In addition, since oxygen levels typically drop by a few percentage points while sleeping, it is advisable to measure them during sleep if the resting daytime level is already low (less than 95 percent). With prolonged exertion, persisting low levels of oxygen saturation (less than 88 percent) should be avoided. If the desaturation is likely to be very brief, then the benefits of using oxygen therapy are unknown and should be discussed with a physician. The usual maximum flow that can be delivered is six liters per minute, but it can be supplemented with transtracheal oxygen therapy.


Prednisone

The most commonly used corticosteroid in the United States. Corticosteroids suppress multiple arms of the immune system, work relatively quickly and are among the most reliable medications. Thus, they are often used for initial treatment for ILD. However, they can cause a range of toxicities over time, including mood changes, elevated blood sugar (diabetes), glaucoma, cataracts, water retention, high blood pressure, stomach ulcers, bone thinning, skin thinning, hip bone necrosis (loss of blood supply to the hip bone), infections and weight gain. Dosing of prednisone for interstitial lung diseases is not standardized, but most experts start with doses ranging from 30 to 60 mg daily with reductions (tapering) over time. Individuals using more than 15 to 20 mg of prednisone daily should generally also take a prophylactic antibiotic to help prevent infections and consider steps to prevent bone loss.


Pulmonary rehabilitation

An individualized exercise regimen designed to improve exercise limitations. While there are no exercises that improve the lungs themselves, pulmonary rehabilitation decreases the amount of work that the lungs must do by improving muscle efficiency and cardiovascular fitness. In ILD patients, pulmonary rehabilitation has been shown to improve the ability to do activities, reduce shortness of breath and improve the quality of life in those with idiopathic pulmonary fibrosis. It is used for other ILD for its anecdotal evidence, although there are little or no data supporting its use in those diseases. We typically prescribe pulmonary rehab for 36 sessions over 12 weeks, followed by exercises being continued at home.


Six-minute walk test

A timed walk that measures exercise capacity and whether oxygen levels in the blood are adequate during exertion. In some ILDs, the six-minute walk test has been shown to correlate with the severity of pulmonary function tests, the likelihood of disease progression and risk of death. However, it does not usually shed much light on breathlessness during activity, or assessing whether breathing tests are deteriorating over time. It is most useful as a research tool.


Spirometry

A pulmonary function test where a maximal effort is used to exhale in order to measure lung capacity (forced vital capacity, FVC). Airflow obstruction is commonly seen in several of the ILDs, including sarcoidosis, hypersensitivity pneumonitis, lymphangioleiomyomatosis, and bronchiolitis caused by connective tissue diseases (such as rheumatoid arthritis).


Surgical lung biopsy

A biopsy (taking a tissue sample) performed in the operating room by a surgeon, either with an incision or using a videoscope that is inserted through small (approximately 1 cm each) incisions in the chest wall. A surgical lung biopsy carries higher risks than other diagnostic procedures, and may not be necessary when the chest CT or other testing is sufficiently definitive. Some ILDs (UIP, NSIP, chronic hypersensitivity pneumonitis, COP) are more likely to require surgical lung because of inconclusive CT scans and the fact that they cannot typically be diagnosed definitively with bronchoscopy.


Transtracheal oxygen therapy (TTOT)

A form of oxygen delivery in which a small hole (the diameter of a wet spaghetti noodle) is made in the neck to place a tube into the airway that is connected to a normal oxygen delivery system. It can be used alone or in conjunction with standard nasal oxygen to increase overall oxygen delivery. Typical flow rates with TTOT are lower than with nasal oxygen since the delivery is more efficient. TTOT catheters require some daily cleaning, and should be avoided in individuals using blood thinners or when there is substantial mucus. Many people prefer TTOT as it is less obtrusive and can be concealed better.


Vasculitis

A group of disorders characterized by inflammatory destruction of blood vessels. Both arteries and veins can be affected.
The different types of vasculitis are classified by their underlying cause, if it can be identified, or according with the size of vessel affected: large, medium or small-vessel vasculitis.

Symptoms of vasculitis are many, often making it difficult to make the diagnosis. They may include chronic fever, weight loss, raised rash, muscle and join pain, numbness or tingling sensation over extremities, nose bleeds, bloody cough, shortness of breath, abdominal pain, bloody stools and dark urine. Blood tests to confirm the presence of ANCA antibodies can be helpful in making the diagnosis.

Sometimes, vasculitis affects the lungs seriously, and poses a high risk of mortality without adequate treatment. Treatment is based on the severity of the disease. Less severe cases may be treated with medications as methotrexate. Severe disease requires an aggressive approach with strong medications as cyclophosphamide. In the majority of cases, patients will be placed on long-term therapy to avoid relapse.