Dr Gillani"s Blog: A 45 year old man presents to your clinic with complaints of increasing shortness of breath over last 6 months.

A 45 year old man presents to your clinic with complaints of increasing shortness of breath over last 6 months.

He also gets occasional episodes of dry cough when walking or going up stairs.

On examination :

He is afebrile / normal Temperature and oxygen saturation is 89 Percent on air .

You organise a chest X ray which is given below:

1. What are findings on chest X ray?

2. What is the most likely Diagnosis?

3. What are the causes of this condition?

4. What further investigations would you like to perform?

5. How would you manage this condition?

Answers given in comments section.

1. What are findings on chest X ray?

Increased interstitial markings” on chest x-ray because of the inflammation, swelling or scarring of the interstitium makes the tissue denser so that it is now visible as white “interstitial markings” on the x ray or scan.

2. What is the most likely Diagnosis?

Interstitial Lung Disease (ILD) Interstitial Lung Disease (ILD) refers to a group of problems in the lung that affects the “interstitium”.

The interstitium refers to the tissue area in and around the wall of the airsacs (alveoli) of the lung area where oxygen moves from the alveoli into the the capillary network (small blood vessels) that covers the lung like a thin sheet of blood.

Once the oxygen crosses the intersitial space it enters the bloodstream and is delivered to the vital organs of your body. ILDs cause this interstitial space to become inflammed or scarred making it more difficult for oxygen.

3. What are the causes of this condition ? Causes of ILD

There are many causes of ILDs and they can be categorized as pictured above into three broad, main groups:

1) Exposure-Related,

2) Autoimmune-Related and

3) Idiopathic, or with no known cause. Exposure-Related (Hypersensitivity Pneumonitis or 'HP'):

Inhalation-related:

There are more than 300 causes of inhalational exposure-related ILDs.

These exposures can be experienced in the workplace, at home, while doing hobbies, or during travel.

Some of the more common exposures are mold, birds in or around the home, bedroom, or backyard, farming/agricultural work, indoor hot tubs, standing water, down feathers, and cleaning chemicals.

Historically, coal miners (pneumoconiosis) and exposure to asbestos (asbestosis) were significant problems, but these exposures have lessened due to the use of protective respiratory equipment.

Medication-related: Some of the medications that can cause an inflammatory reaction in the lung include amiodarone, methotrexate, some chemotherapy agents used to treat cancers, and nitrofurantoin (sulfa drugs).

Autoimmune-Related ILDs (Nonspecific Interstitial Pneumonitis – NSIP): Autoimmune–related ILDs tend to occur more often in women and in persons under the age of 65. Idiopathic ILDs (no known cause):

Idiopathic Pulmonary Fibrosis (IPF) is the most common ILD that has no known cause.

4. What further investigations would you like to perform? Radiology evaluations:

A Computed Tomography (CT) scan of the chest is the most sensitive diagnostic tool for ILD. The best assessment is with a high resolution CT scan (HRCT) which provides thin slices (1-2 mm thick) of the lungs in order to closely evaluate the lung tissue changes. Chest X-Rays may show ILD but are not diagnostic. Breathing tests:

Pulmonary Function Studies (PFTs) are used to evaluate the severity of the lung problem. PFTs evaluate the ability of the lung to:

move air in and out of the lungs (spirometry- Forced Vital Capacity (FVC), Forced Expiratory Volume in 1 second (FEV1). Lung Tissue Sampling: If the decision is made that a lung tissue sample (lung biopsy) is needed in order to make a diagnosis, there are a few different options:

Transbronchial Biopsy (TBBx) with Bronchoalveolar Lavage (BAL) can be done to obtain a very small piece of lung tissue and also a sample of a ‘washing’ of sterile water in and out of the lungs. It is performed as an outpatient procedure.

Blood Tests:

There are different types of blood tests that may be ordered to assist in making an accurate diagnosis Serology: These are blood tests that look for antibodies (proteins made by your immune system) in blood to determine if patient have a type of autoimmune disease (lupus, slceroderma, Sjögren's sydrome, polymyositis/ dermatomyositis, mixed connective tissue disease).

Examples of these blood tests include:

Rheumatoid Factor: rheumatoid arthritis

ESR: estimated sedimentation rate

CRP: C-reactive protein reflecting a response to inflammation

ANA: Antinuclear Antibodies

anti-dsDNA: anti-double stranded DNA

anti-SSA (Ro) and anti-SSB (La): anti Smith antigens

Anti-JO-1

ANCA: antineutrophil cytoplasmic antibodies

anti-Scl 70: scleroderma

5. How would you manage this condition?

Pharmacological Treatment to suppress further inflammation includes medications such as : Corticosteroids /Prednisolone , Azathioprine, Cyclophosphamide, Hydroxychloroquine and Methotrexate.

Anti-Fibrotic (anti-scarring) Agents include medications like Nintedanib (Ofev®) and Pirfenidone (Esbriet®, Pirfenex®, Pirespa®.

Non-pharmacological options include : Pulmonary Rehabilitation , oxygen and Palliative Treatment.

Pulmonary Rehabilitation (PR) is a structured exercise and education program for people with chronic lung diseases, including ILD, with the goal of maximizing a patient’s ability to maintain activity, decrease breathlessness and fatigue, and improve quality of life.

Typically, pulmonary rehabilitation will include exercise training (aerobic, strengthening, flexibility); breathing exercises; anxiety, stress, and emotional management strategies; nutritional counseling; disease education; medication education, and other components.