FIBROSING PULMONARY DISEASE
& OCCUPATIONAL LUNG DISEASE
(PULMONARY INTERSTITIAL FIBROSING DISEASE)
2017
BY
DR. DANJUMA SULAI, OON
MBBS, MSc. Com Hlth (Lon) FRCP (Lon) FMCP (Nig.)
email: danjumasulai@yahoo.com
- A clinical state in which the Pulmonary parenchyma
at the respiratory bronchioles and alveolar region
are being replaced with fibrous tissue, following
inflammatory cells and exudates. Alveolar air-sacs
are obliterated with thickened wall and fibrous
tissue.
- The vascularity of the respiratory acini distorted
causing progressive loss of alveoli - capillary gas
exchange.
These results in:
* It is the form of Chronic Restrictive Pulmonary
Disease (CRPD) in contrast to Chronic Obstructive
Pulmonary Disease (COPD).
* There are minor obstructive airways features but poor
gas transfer is the predominant outcome, with
progressive exceptional dyspnea, Respiratory failure,
Pulm. Hypertension that could result in Cor pulmonale.
Classified into: Intrinsic, Extrinsic, and Rare Causes – Neuro
fibromatosis, Pulm. Haemosiderosis
a) Intrinsic Causes:
i) Cryptogenic Fibrosing Alveolitis
ii) Sarcoidosis
ii) Secondary to defined collagen disorders like
Rheumatoid disease, systemic lupus Ethrythromatosis
(SLE) ARDS.
i) Sarcoidosis
Multi – system disease- histologically granulomatous without caseation
and AFB unlike TB
– 30% Fibrosing Lung Disease – insidiously
- Hilar Lymph nodes enlargement,
- Cough, dyspnea with exertion, finger clubbing,
- granuloma in liver, phalanges, Parotids, nose
- Acutely with glaring involvement of uveitis, arthritis,
erythema nodosum nodules on shins, fever,
Nephrocalcinosis. Cause not known.
- Chronic Beryllium poisoning mimicks sarcoidosis, but two
Not related.
a) Diagnosis
- Tissue biopsy transbronchial
- Depressed tuberculin reaction
- CXR
b) Management –use Corticosteroid
ii) Cryptogenic Fibrosing Alveolitis (CFA) also called
Idiopathic Pulmonary Fibrosis.
- Finger Clubbing accompanying increasing
dyspnoca with dry cough
- Cause not known –
- Shrunken lungs Inspirational Crepts elevated diaphragm,
Diffuse Pulm. Opacities lower zones, could become
honeycomb in appearance.
- Diagnosis – based on clinical history, Chest X-ray; High
Resolution CT, Restrictive Lung Function Tests with reduced
Gas Transfer Factor, Last result Biopsy.
Management
- Prednisolone with azathioprane being used but Mortality
high and survival beyond 5 years virtually nil!
These are dust; and chemical fumes – chronic
exposure and inhalation of them is during occupation using
those items. These Pulmonary diseases are often called
‘Occupational Lung Disease.
i) Organic Dust/Chemicals
a) Bird Fancier Lung Disease consequent to Bird
droppings, fancying by elderly ladies, now
open battery, non-caged poultry, feeding of
chickens, cockerels, their droppings a long
with even saw dust is inhaled.
b) Byssinosis – Cotton products in Textiles, thread
and gowns industries. Starts is “Monday Fever”
with Lassitude , bronchitis – then progress into
shortness of breath.. Improve with removal from
the Dust (Cotton).
Extrinsic cause –(CFPD)
ii) Non – Organic Cause (Minerals)
a) Silicosis – sand, quarts, granite/quarry work; tunnel
digging – mothers crushing stones/gravel for sale. Silicon
dioxide. Silica is very Fibrogenic into Hard nodules, non-
remitting.
b) Pneumoconiosis – Coal mining. The Coal Mines of Enugu
has its tell tale in the Clinical Lives of those who worked
then and are now in their over 60 -70 years. Tarry block
sputum associated with chronic bronchitis nodules
present (caplans syndrome)…./associated with Rheumoid disease.
- Nodules in Lungs, small sizes early phase. Could
regress fully if leave exposure.
- Multi- nodular, cavitating upper lobes, interstitial
fibrosis progressive. None remitting.
crocidolite(blue), amosite (brown)- at mining,
demolition, ship breaking, break-pads, boiler
lagging jobs.
- Asbestos disease also manifests as pleural
effusion, pleural fibrosis, pleural mesothelioma,
- Benign pleural plaques, carcinoma bronchus,
carcinoma larynx.