Pulmonary interstitial fibrosis (PF), short for “diffuse pulmonary interstitial fibrosis”, is a group of heterogeneous diseases caused by inflammation of the alveolar wall, followed by the formation of a large amount of fibrous connective tissue in the interstitial lung and disorders of lung structure. In recent years, some studies have reported this disease as “shortness of breath”, “asthma”, “cough”, “lung impotence”, ” lung paralysis”, “lung distension”, etc.
Etiology
Diffuse chronic interstitial lung fibrosis is an inflammatory disease of the interstitial lung caused by a variety of causes, involving mainly the interstitial lung, but also the alveolar epithelium and pulmonary vasculature. The etiology is either clear or unknown.
Clear causes include inhalation of inorganic dusts such as asbestos and coal; organic dusts such as mold and grass dust, cotton dust; gases such as soot and sulfur dioxide; viral, bacterial, fungal and parasitic infections; drug effects and radiation damage. Secondary to autoimmune diseases such as lupus erythematosus. This disease belongs to the category of “cough”, “asthma” and “lung fistula” in Chinese medicine.
Clinical manifestations
The onset of the disease is insidious, with progressive exacerbation. It is characterized by progressive shortness of breath, dry cough with little sputum or a small amount of white mucous sputum, and respiratory failure with mainly hypoxemia in the late stage. On examination, the thoracic respiratory motion is reduced, and fine wet rales or twanging sounds can be heard in both lungs. There are varying degrees of cyanosis and pestle-like fingers. Signs of right heart failure may be present in the late stage.
Diagnosis
1.Progressive shortness of breath, coughing, wet rales or twisted sounds in the lungs.
2, X-ray examination: early hairy glassy, typical changes diffuse linear, nodular, cloudy, reticulated shadows, lung volume reduction.
3, laboratory tests: ESR and LDH are seen to be increased, generally without special significance.
4, pulmonary function tests: reduced lung volume, reduced diffusion function and hypoxemia are seen.
5, lung tissue biopsy provides pathological basis. This disease should be distinguished from asthmatic bronchitis.
Laboratory tests are non-specific changes and may include accelerated sedimentation, increased blood lactate dehydrogenase and increased gammaglobulin; 10%-26% of patients are positive for rheumatoid factor and antinuclear antibodies.
Diagnostic criteria
The diagnosis is based on clinical features, chest X-ray, pulmonary ventilation and diffusion function, pathological biopsy, and exclusion of other known causes of ILD. there are 2 confirmatory criteria based on the presence or absence of surgical lung biopsy findings.
(i) Confirmation criteria I.
1. Surgical lung biopsy shows histological changes consistent with common interstitial pneumonia.
2. The following conditions are also present.
(i) Exclusion of other known diseases that can cause ILD, such as drug poisoning, occupational environmental exposure and connective tissue disease.
(ii) Restrictive ventilation dysfunction with decreased diffusion function on pulmonary function tests.
(③ Conventional X-ray chest film or HRCT shows predominantly reticular changes in both lower lungs and subpleural distribution or with honeycomb lung, which may be accompanied by a small amount of ground glass shadow.
(B) Confirmation criteria II: In the absence of surgical lung biopsy, all 4 of the following major indicators and 3 or more minor indicators need to be met.
1. Primary indicators.
①Excluding known causes of ILD, such as toxic effects of certain drugs, history of occupational environmental exposure and connective tissue disease.
(ii) Abnormal lung function performance, including restrictive ventilation dysfunction [reduced lung volume (VC) with normal or increased FEV1/FVC] and/or impaired gas exchange.
(iii) HRCT of the chest showing reticular changes or cellular lung with predominantly bilateral lower lung and subpleural distribution, which may be accompanied by a small number of ground glass shadows.
④TBLB or BALF examination does not support the diagnosis of other diseases.
2. Secondary diagnostic conditions.
① Age >50 years.
②Progressive dyspnea with insidious onset or without clear cause.
③ duration of disease ≥ 3 months.
(④Aspiratory Velcro woven worm can be heard on auscultation of both lungs.
Medical theory
Pulmonary paralysis has the pathogenetic evolution of the external evil that has not ceased to be present and is internally surrendered to the lung (long-standing disease into the lung). This is quite consistent with modern medicine regarding secondary interstitial lung lesions. The pathogenesis of pulmonary paralysis is characterized by deficiency of positive energy and paralysis of the lung channels. Because of this, interstitial lung fibrosis is often named “pulmonary paralysis”, emphasizing that the pathogenesis is characterized by paralysis of the lung channels; on the other hand, it reminds us that rheumatic immune diseases, especially rheumatoid arthritis (paralysis), scleroderma and dermatomyositis (dermatomyositis), can also cause interstitial lung fibrosis (secondary interstitial lung fibrosis), forming “Pulmonary paralysis”.
”Lung impotence” refers to a disease caused by weakness of the lung and the inability of the lung to distribute Qi and fluid, with coughing and salivation as the main symptoms. Interstitial lung fibrosis is called “lung impotence” mainly for the following reasons.
(1) In terms of morphology, the volume of both lungs shrinks in the middle and late stages of pulmonary fibrosis, and the total lung volume, lung volume, residual air volume and tidal volume are all significantly reduced, which is consistent with the original meaning of “pulmonary impotence”.
(2) In terms of pathogenesis, the basic pathogenic features of pulmonary impotence are heat in the lungs, deficiency of fluid and blood, and loss of moistening. Lung fibrosis is persistent, and the pathogenesis is transformed from qi to blood, from lung to kidney, with deficiency of both lung and kidney, and deficiency of qi and blood, with deficiency of the lung and kidney not being honored, “deficiency of the lung is impotence”.
(3) In terms of clinical characteristics, interstitial fibrosis of the lung is prolonged and persistent, with “the initial disease of qi knotting in the meridians, and the prolonged blood injury into the lao”, and in the advanced stage, the lung is honeycombed (lattice-like changes), and even the lung is destroyed and lung function is lost. This is exactly like the lung impotence disease of the lung lobe atrophy and weakness, delayed and repeated, the characteristics of long-term treatment.
In summary, “pulmonary paralysis” and “pulmonary impotence” are both TCM diagnoses of pulmonary interstitial fibrosis, with pulmonary paralysis referring to the lung being paralyzed by evil, with Qi and blood blockage and stagnation of the ligaments, in terms of the actuality of evil; and pulmonary impotence referring to the lung being atrophied and not used, with Qi and blood not being filled and the ligaments being deficient and not honored, in terms of the original deficiency. Both reflect the different stages of the development of pulmonary fibrosis. However, pulmonary paralysis and pulmonary impotence can affect each other under certain conditions. Generally speaking, there is a clinical evolution of pulmonary fibrosis from pulmonary paralysis to pulmonary impotence, which means that the actuality leads to deficiency. However, “the place where deficiency exists is the place where evil is retained”, and impotence is commonly associated with paralysis of the lung channels. Pulmonary fibrosis can often be seen as a complex pathology of impotence within paralysis (deficiency due to actuality) and paralysis within impotence (actuality due to deficiency), i.e., “lesions of varying severity, old and new lesions coexist”, which should be examined and identified in detail.
Pathology
The complex pathogenic factors stimulate various cytokines, histamines, proteases, oxidants, etc. to form immune complexes and alveolar macrophages, neutrophils, lymphocytes and fibroblasts to collect in the interstitial lung, resulting in interstitial lung inflammation, resulting in interstitial lung fibroblasts and excessive collagen deposition, scarring and destruction of lung tissue, and finally interstitial lung fibrosis. This disease is chronic and progressive, and is a difficult and serious disease of the lung system. The causes of pulmonary hypertension in late stages are different from those of hypertension. It is generally believed that the cause of pulmonary hypertension is spasm of the small pulmonary arteries, but it is presumed from the clinical presentation of the patient that it is due to obstruction of pulmonary capillary passage. The alveolar area of a normal person has a total area of about 100 square meters, about the size of a tennis court, and is responsible for the exchange of oxygen and carbon dioxide throughout the body. Its capacity to pass and the capacity to pass capillaries of other tissues and organs throughout the body should be in balance. Whether it is alveolitis or fibrosis, this diffuse damage decreases the permeability of the capillaries of the alveoli, and the blood pumped from the right ventricle cannot pass smoothly through the alveoli to complete gas exchange, breaking the balance between the small and large circulating blood volumes, resulting in increased pulmonary artery pressure, which can develop into right ventricular failure in severe cases.
Symptoms
About 15% of IPF cases have an acute course and are often detected by visits for upper respiratory tract infections, with progressive dyspnea increasing and death from respiratory and circulatory failure within 6 months. The vast majority of IPF cases are chronic (there may be intermediate subacute forms), and although they are called chronic, the average survival time is only 3.2 years. The chronic form does not appear to evolve from the acute form, and the exact relationship is not known.
The main symptoms are.
(i) dyspnea Exertional dyspnea with progressive worsening, shallow and rapid breathing, with possible nasal movement and participation of the auxiliary muscles in breathing, but mostly without telangiectatic breathing.
②Cough and sputum There is no cough at the early stage, and later there may be dry cough or a small amount of mucus sputum. It is easy to have secondary infection and mucopurulent sputum or pus sputum, and blood sputum is occasionally seen.
(③) Systemic symptoms There may be wasting, weakness, loss of appetite, joint pain, etc., which are generally rare. Acute type may have fever.
Common signs
①Difficulty in breathing and cyanosis.
②Thoracic dilatation and decreased diaphragm mobility.
③Velcro rales in the lower and middle part of both lungs with certain characteristics.
④Pestle-like toes.
⑤ End-stage respiratory failure and right heart failure corresponding signs.
Features
Interstitial lung fibrosis This type is the most common in IIP (accounting for about 65%) and is more frequent in adults over 50 years of age, with about 2/3 of patients older than 60 years of age and more men than women. The clinical manifestations are dry cough and dyspnea. Inspiratory bursting sounds can be heard in most patients, most obvious at the base of both lungs, and pestle fingers can be seen in more than one-third of patients. Pulmonary function abnormalities are mainly moderate to severe restrictive ventilatory dysfunction and/or diffusion dysfunction. Laboratory tests lack characterization, with 10% to 25% of patients having positive serum antinuclear antibodies (ANA) and rheumatoid factor (RF).
Infectiousness
Most pneumonia is not contagious. Bacterial infections in adults with pneumonia are most commonly caused by Streptococcus pneumoniae. Other pathogens include anaerobes, Staphylococcus aureus, Haemophilus influenzae, Chlamydia pneumoniae, Chlamydia psittaci, Chlamydia trachomatis, and other gram-negative bacilli, among many others. These pathogens may be transmitted through contact between people, or through contact between people and objects, but even if you are infected with these pathogens, you will not get pneumonia if your immune system is sound. It is often when the body’s resistance decreases that pathogens can take advantage of the situation and make people sick.
Precautions
1, to ensure that there is enough rest, but also pay attention to keep warm, to avoid cold, to prevent various infections. Pay attention to climate change, especially in winter and spring, when the temperature changes drastically, add and remove clothes in time to avoid aggravating the disease after getting cold.
2, to have a comfortable living environment. The room should be quiet, keep clean and hygienic, the air should be fresh, moist and circulating, avoid smoke, perfume, air fresheners and other stimulating factors with strong odors, also avoid inhaling too cold, too dry, too wet air.
3, diet, diet should be light, easy to digest, mainly liquid or semi-liquid, eat more fruits and vegetables, drink more water, avoid spicy, sour, hemp, spicy, fried food and eggs, fish, shrimp and other foods that can easily induce asthma. Do not eat stimulating food. In general, the diet should be characterized as diverse, reasonably matched, nutritious, appropriately proportioned, and suitable for digestion and absorption.
4.Mentally, you should maintain a happy and optimistic mood and prevent mental stimulation and excessive mental tension. This requires you to have an open-minded and cheerful attitude to life, that is, to maintain a happy spirit, we must cultivate the idea of “contentment and happiness”, not excessive pursuit of fame, fortune and enjoyment to appreciate the truth that “more than the top is not enough, more than the bottom”, so that you can feel life and Psychological satisfaction. Keep your spirit happy and arrange your daily life in a colorful way.
5. Stay away from exogenous allergens such as: some flowers and plants (especially for those who are allergic to pollen), bedding filled with allergy-prone items such as feathers or old lint, pillows, birds, animals (pets or experimental breeders), wood (redwood dust, cork processing), sugar processing, mushroom farming, cheese, wine processing, moldy straw exposure, water sources (hot water pipes, air conditioners, humidifiers, sauna baths) and agricultural pesticides or herbicides, etc.
Treatment suggestions
1.Western medicine treatment
(1) Hormone therapy: prednisone 30-40 mg orally in 2-3 doses, gradually reducing to maintenance dose of 5-lO mg once a day.
(2) Treatment of complications: anti-infective treatment, select antibiotics according to the pathogenic bacteria.
(3) Bronchodilators: aminophylline, salbutamol, etc.
(4) Oxygen therapy: applicable to patients with advanced stage.
2.Chinese medicine treatment
(1) Lung Qi deficiency: low cough and wheezing, easy fatigue, sweating and fear of wind, easy to catch cold, light tongue with white fur and weak pulse.
Treatment: Tonify lung qi, stop coughing and stabilize asthma.
Herbs: 30 grams of raw Astragalus membranaceus, 10 grams of raw Atractylodes macrocephala, 10 grams of almonds, 10 grams of asters, 10 grams of dried flowers, 6 grams of windproof, 15 grams of prunus ginseng, 6 grams of roasted ephedra, 6 grams of licorice.
Chinese patent medicine: Yu Ping Feng granules.
(2) Qi and Yin deficiency, phlegm blocking the lung: dry cough without or with little phlegm, shortness of breath, increased by movement, fatigue, dry mouth, dry throat, irritable heat in the five hearts, lumbar soreness and knee weakness, light red tongue, thin white or little moss, thin smooth or weak pulse.
Treatment: Tonifying the lung and nourishing the kidney, resolving phlegm and activating blood.
Herbs: 15 grams each of prunus ginseng, rehmannia, shamrock, yam, poria, 30 grams of maitake, 10 grams of schisandra, 10 grams of tannin, 10 grams of zedoary, 30 grams of raw astragalus, 30 grams of lily, 15 grams of walnut, 30 grams of salvia, 10 grams of aster, 6 grams of Chuanbei powder (for dosing).
Prepared Chinese medicines: raw pulse drink oral liquid, Liu Wei Di Huang Wan.
(3) Spleen and kidney yang deficiency, internal blockage of blood: cough and asthma are weak, increased when moving, more breathing and less inhalation, swelling of lower limbs, cold form and cold limbs, gray face and purple lips, light fat tongue, thin white fur, sunken and weak pulse.
Treatment: Strengthening the spleen, warming the kidney, resolving phlegm and activating blood circulation.
Herbs: 10 grams of cooked spleen, 5 grams of cinnamon, 15 grams of rehmannia, 15 grams of shamrock, 15 grams of yam, 15 grams of poria, 10 grams of dandruff, 0 grams of zedoary, 30 grams of raw astragalus, 30 grams of danshen, 10 grams of aster, 10 grams of almond, 10 grams of dilong, 10 grams of Xianling spleen.
Prepared Chinese medicine: Ginseng and Spleen Pill, Jin Kui Kidney Qi Pill.
3.Chinese medicine treatment
The magnetic superimposed regulation of immunity through the theory of diagnosis and treatment, not a single Chinese medicine treatment, for each patient’s physical characteristics and symptom differences, according to the individual, depending on the evidence of the selection of different acupuncture points, prescriptions, the development of different treatment plans, mainly through acupuncture, cupping, fumigation, Chinese medicine compress and other techniques combined treatment.
Prevention and maintenance
1, pay attention to avoid cold and keep warm, prevent cold and flu.
2.Avoid contact with foreign bodies with clear causes.
3, pay attention to diet and nutrition.
4.Ginseng, mealybug, Chuanbei powder, safflower, cordyceps in appropriate amount, powder human capsule, take in appropriate amount, or consume 2-3 walnuts daily. The body can be seen with reduced thoracic respiratory movement, and fine wet rales or twang sounds can be heard in both lungs. There are varying degrees of cyanosis and pestle-like fingers. Signs of right heart failure may appear in the late stage.
The danger of pulmonary fibrosis
1, lung infection, respiratory failure: Patients with pulmonary fibrosis have a weakened lung function and reduced lung immunity, and most of them apply glucocorticoids and immunosuppressants during the conventional treatment, which increases the incidence of lung infection. When the lung infection is not better controlled, respiratory failure can be induced.
2, pulmonary heart disease, heart failure: patients with pulmonary fibrosis chronic hypoxia, progressive pulmonary hypertension, often combined with right ventricular hypertrophy and pulmonary heart disease. Left heart failure is also common and is associated with ischemic heart disease.
3, pulmonary hypertension: Patients with pulmonary fibrosis are mostly associated with chronic hypoxia and carbon dioxide retention causing extensive narrowing of the small pulmonary arteries and increased resistance of the pulmonary circulation, leading to the development of pulmonary hypertension. Pulmonary hypertension is one of the important factors affecting pulmonary fibrosis. Therefore, early detection of the disease should be timely intervention and treatment, which has an important impact on the improvement of the prognosis and the quality of survival of patients.
4, the impact on the respiratory system: when the partial pressure of arterial oxygen drops below 60 mmHg, hypoxemia stimulates peripheral receptors and ventilation increases, reaching a peak when the partial pressure of oxygen is 30-40 mmHg. The respiratory center’s response to ventilation during hypoxemia is much lower than that of carbon dioxide, due to the poor sensitivity of chemoreceptors to hypoxemia. Ventilation increases during chronic hypoxia. When the partial pressure of arterial oxygen is significantly reduced there is an inhibitory effect on the respiratory center and severe hypoxia can cause irregular and tidal breathing. The more severe the hypoxia, the earlier the onset of pulmonary heart disease. Long-term hypoxia will lower the immunity of the body and make it easy for acute respiratory infections to occur.
5, the impact on the circulatory system: hypoxemia through chemoreceptors to sympathetic nerve strong stimulation, acute hypoxia early due to the direct response of the blood vessels and through the influence of neural reflexes, heart rate accelerates, blood pressure increases, cardiac output increases. The increase in cardiac output is an immediate response via chemoreceptors and appears before the increase in catecholamines. As hypoxemia worsens, arrhythmias occur, followed by a decrease in blood pressure, a slowing of heart rate, and a decrease in cardiac output. In chronic hypoxia, cardiac output does not change significantly with respect to the surroundings, but pulmonary vascular resistance increases, leading to increased right heart load and, in the long term, to chronic pulmonary heart disease.