What is a biosimilar
Interstitial Lung Diseases (PID) are a heterogeneous group of lung pathologies that affect the alveolar-interstitial structures and present similar clinical-radiological characteristics.
Causes
The etiological factors involved in the origin of PID are very varied, currently more than 150 causes are known and, despite this, the etiology can only be established in 30-40% of cases.
symptomatology
The most frequent symptoms of the disease are:
- Dyspnea: The sensation of suffocation, in the initial phases, appears with intense effort and as the disease progresses it increases until it occurs at rest.
- Chronic and dry cough: It is not usually accompanied by secretions.
- Peripheral cyanosis: Bluish coloration of the skin and mucous membranes due to lack of oxygenation.
- Finger clubbing or clubbing
But not all symptoms are centered on the respiratory system, it is very important to assess systemic involvement in order to make a correct diagnosis. The most frequent systemic conditions and the PID that produce them are:
- skin involvement
- Erythema nodosum: sarcoidosis, collagen diseases
- Neurofibromas and “café au lait” spots: neurofibromatosis
- Subcutaneous nodules: rheumatoid arthritis, neurofibromatosis, sarcoidosis
- Heliotrope rash: dermatomyositis
- Albinism: Hermansky-Pudlak syndrome
- eye involvement
- Scleritis: lupus, scleroderma, sarcoidosis
- Keratoconjunctivitis sicca: Sjögren's syndrome
- Uveitis: sarcoidosis
- Lacrimal gland enlargement: sarcoidosis
- Musculoskeletal involvement
- Myositis: collagen diseases
- Arthritis: sarcoidosis, collagen diseases
- Bone involvement: histiocytosis X, sarcoidosis
- Neurological affectation
- Sarcoidosis, neurofibromatosis, tuberous sclerosis, collagen diseases
- kidney involvement
- Angiolipomas: lymphangioleiomyomatosis
- Nephrotic syndrome: amyloidosis, lupus
- Glomerulonephritis: collagen diseases
- digestive affectation
- Hepatosplenomegaly: sarcoidosis, histiocytosis X, diseases
- of collagen, amyloidosis
- Chronic Diarrhea: Inflammatory Bowel Disease
- Dysphagia: systemic sclerosis, dermatomyositis/polymyositis
- heart involvement
- Myocardium: sarcoidosis
- Pericardium: collagen diseases
- endocrine involvement
- Diabetes insipidus: sarcoidosis, histiocytosis X
Diagnosis
Establishing a diagnosis of PID is complicated by the initial signs and symptoms of these pathologies are very similar to those of other respiratory pathologies and to each other.
To make a correct diagnosis, a detailed clinical history will be carried out, including:
- Age and sex: Depending on these two factors, it will be more likely that one PID or another will be suffered.
- History of smoking: There are some PID typical of smoking and others that are not.
- Family background
- Environmental exposure, work history and/or Exposure to animals (birds, pigeons): Frequent exposure to one or more of these elements will determine the EIP
- Use of medications: Certain drugs are often a frequent cause of PID
- Previous treatments with radiotherapy
- Existence of some systemic diseases (collagenosis…)
This history will be complemented with a physical examination that includes lung auscultation, observation of the existence of cyanosis and clubbing, as well as laboratory tests and other diagnostic tests such as:
- Diagnostic imaging techniques:
- Chest X-ray: Allows to see the amount of fibrous lung tissue and its distribution
- High Resolution Computed Axial Tomography (HAT)
- Lung function tests:
- blood gases
- pulse oximetry
- Walk test 6 minutes
- Respiratory function tests: They are a basic tool for diagnosis, guide the prognosis, control the evolution of the disease and assess the response to treatment. It must be taken into account that a normal respiratory function test does not exclude the diagnosis of PID. These tests measure:
- Air flow: Spirometry
- lung volumes
- Carbon Diffusion Capacity
- bronchopulmonary lavage
- Lung biopsy: Used for definitive diagnosis
Differential diagnosis
The differential diagnosis has to be made with:
- Heart failure
- Bronchiectasis
- Pneumonia
- carcinomatous lymphangitis
- Pulmonary infiltrates in immunocompromised patients
- Diffuse pulmonary hemorrhages
- Lipoid pneumonia
- Miliary tuberculosis and bacillus Calmette-Guérin miliary disease
Complications
The most frequent complications are:
- Respiratory insufficiency
- Respiratory infections
- Pulmonary hypertension
- Lung cancer
- Pulmonary embolism
- Pneumothorax
- mycetoma
Treatment
Pharmacological
The efficacy of various antifibrotic drugs is currently being investigated for use in the treatment of respiratory involvement, but it must be remembered that not all PID evolves into fibrosis.
In addition to treating the respiratory condition, each type of PID will have a specific treatment for its systemic alterations...
In the most serious cases, as long as the patient is under 65 years of age, the only treatment option is lung transplantation, but this has many contraindications to take into account.
Oxygen
Its use will be prescribed in patients when blood oxygen levels are less than 50 mmHg or oxygen saturation less than 90%.
Although its use cannot stop the progress of the disease, it can make breathing easier and allow the patient to continue with his active life for as long as possible, in addition to reducing complications and associated problems.
Associations
AFEFPI- Association of Relatives and Patients of FPI and FPF
(Idiopathic Pulmonary Fibrosis)
Place of Cobas, 3 – Os Tilos
15894 THEO (A CORUNA)
Phone: 687 468 205
Contact: afefpi@gmail.com
Website: www.orpha.net/consor/cgi-bin/index.php
Idiopathic Pulmonary Fibrosis Association
Phone: 639 072 908
Contact: airetxp@hotmail.com
AELAM- Spanish Association of Lymphangioleiomyomatosis
Telephone: 986 129 340/ 693 639 362
Contact: aelam@aelam.org
Website: www.aelam.org
CHE- Association Against Langerhans Cell Histiocytosis
Pozuelo de Alarcon, Madrid
Phone: 639 139 192
Contact: rsuarez@histiocytosis.org
Website: www.histiocytosis.org
