interstitial lung diseases

Question 1

ILD definition and causes?

Answer 1

Definition:
Interstitial lung disease (ILD) is an umbrella term used for a large group of diseases that cause scarring (fibrosis) of the lungs. The scarring causes stiffness in the lungs which makes it difficult to breathe and get oxygen to the bloodstream. Lung damage from ILDs is often irreversible and gets worse over time.

Causes of interstitial lung disease:
1- Idiopathic interstitial pneumonias
A-Idiopathic pulmonary fibrosis (IPF) or (hamman –rich syndrome)
is a fulminant form of lung injury, It is an interstitial lung disease characterized by rapid onset of respiratory failure, similar to acute respiratory distress syndrome (ARDS) with diffuse alveolar damage , in a previously healthy individual without a history of lung disease. It is not associated with cigarette smoking
B-Acute interstitial pneumonia
C-Cryptogenic organizing pneumonia

2- Occupational and environmental factors
Inorganic dust (pneumoconiosis ):
-Crystalline silica
-Silicates (asbestos, talc, kaolin, mica)
- Beryllium
- Bauxite
- Antimony
- Coal
- Graphite
- Cobalt
- Iron oxides
Organic dusts:
-Hypersensitivity pneumonitis (extrinsic allergic alveolitis ) e,g Farmer’s lung
- Microorganisms (actinomycetes, aspergillus )
- animals (bird bloom, small mammal protein)

3- Collagen diseases:
-Rheumatoid arthritis
-Systemic sclerosis
-Systemic Lupus erythematosus
-dermatomyositis

4- Toxic fumes and vapors:
-Oxygen
-Chlorine
-Nitrogen oxide
-Sulphuric acid
-Heavy metals
-Lipid

5- Drugs:
-B blockers
-Methotrexate
-Cytotoxics
-Nitrofurantoin
-Sulphasalazine
-Salicylates
-Gold
-Penicillamine

6- Vasculitis
-Churg-strauss syndrome
-Polyarteritis nodosa
-Wegener’s granulomatosis
-Idiopathic pulmonary fibrosis (cryptogenic fibrosing alveolitis).
7- others
-radiotherapy
-Sarcoidosis
-Histiocytosis x

Question 2

ILDs diagnosis and Treatment

Answer 2

Diagnosis
1- Symptoms
-Gradual progressive shortness of breath on exertion may be the only symptom
-As disease progress dyspnea occurs at rest
-Dry cough-fatigue .
2- Signs
General:
-Digital clubbing
-May cyanosis in advanced cases.
Local: (inspiratory crackles that not resolved after cough).

Investigations
1-Lab
-Eosi drug reaction
-Lymphopenia in sarcoidosis
-May elevated calcium in sarcoidosis
2-Arterial blood gases
Hypnophilia inpoxemia
Normo or decrease pco2
Rare increase pco2
3- Chest radiography [X-ray ] and chest CT.
-Reticular or nodular pattern or mixed pattern
-mostly at lower lung zones initially, and diffuse when the disease is progressed.
- Honeycombing with cystic changes in progressed disease
4- Pulmonary function testing:
-To assess severity of the disease.
-Mostly showing a restrictive pattern of diseases but in some cases produce obstructive pattern as sarcoidosis
5- Bronchoscopy and bronchoalveolar lavage:
Total and differential cell counts (with particular attention to eosinophilic count).
6- Transbronchial lung biopsy:
7- Surgical lung biopsy (thoracoscopy guided and open lung biopsy) for pathological examination.

Complications:
-Acute exacerbation of lung fibrosis
-Pulmonary hypertension and cor pulmonale
-Bronchiectasis.
-Respiratory failure.
-Thromboembolic disease.
-Lung cancer

Treatment:
(1) Supportive (oxygen, pulmonary rehabilitation).
(2) Treatment of underlying cause if known.
(3) Corticosteroid treatment.
In case of IPF give prednisone 1mg/kg/day in addition to cytotoxic drugs as cyclophosphamide
(4)Antifibrotic drugs (e.g. pirfenidone)
(5) Treatment of complications.
(6) Lung transplantation.
(9) oxygen therapy

Question 3

Sarcoidosis definition and C/P

Answer 3

Definition
Is a (granulomatous disease) in any part of the body 4 most commonly the lungs and lymph nodes. But it can also affect the eyes, skin, heart and other organs.

The cause of sarcoidosis is unknown
But Some research suggests that infectious agents, chemicals, dust and a potential abnormal reaction to the body’s own proteins (self-proteins) could be responsible for the formation of granulomas in people who are genetically predisposed.

1-General symptoms
Sarcoidosis can begin with these signs and symptoms:
*Fatigue
*Swollen lymph nodes
*Weight loss
*Pain and swelling in joints, such as the ankles
2-Lung symptoms
*Sarcoidosis most often affects the lungs and may cause lung problems, such as:gradual progressive dyspnea
*Persistent dry cough
*Central cyanosis ,clubbing
*Wheezing
*Chest pain
3-Liver symptoms
*Hepatomegaly and jaundice
4-Skin symptom as Erythema nodosa:
*A rash of red or reddish-purple bumps, usually located on the shins or ankles, which may be warm and tender to the touch
5-Eye symptoms
*Blurred vision,Eye pain
6-Neurological manifestations
7-Anemia

Question 4

Sarcoidosis diagnosis and Treatment

Answer 4

Diagnosis
*Complete Blood count
*urine analysis
*kidneys and liver function
-Chest X-ray
Chest radiograph changes are divided into four stages:
*bihilar lymphadenopathy
*bihilar lymphadenopathy and reticulonodular infiltrates
*bilateral pulmonary infiltrates
*fibrocystic sarcoidosis typically with upward hilar retraction, cystic and bullous changes
-Computerized tomography (CT) scan
-Lung (pulmonary) function tests
-Electrocardiogram (ECG or EKG) to detect heart problems and monitor the heart’s status
-Eye exam to check for vision problems that may be caused by sarcoidosis
-magnetic resonance imaging (MRI) if sarcoidosis seems to be affecting heart or central nervous system
-Biopsies
*Non caseating granuloma
*Angiotensin –converting enzyme is elevated
*Elevated serum calcium

*Treatment:
-Corticosteroids. These powerful anti-inflammatory drugs are usually the first line treatment for sarcoidosis. In some cases, corticosteroids can be applied directly to an affected area 4 via a cream to a skin lesion or drops to the eyes.
-Medications suppress the immune system. Medications such as methotrexate and azathioprine Hydroxychloroquine. Hydroxychloroquine (Plaquenil) may be helpful for skin lesions and elevated blood-calcium levels.
-Tumor necrosis factor-alpha (TNF-alpha) inhibitors
-Oxygen therapy
-Lung transplantation