Idiopathic Fibrosing Alveolitis (Hamman-Rich Syndrome)

LV Hamman, Amer. Physician, 1877-1946; AR Rich, Amer. Physician, was born in 1893; synonyms: idiopathic pulmonary fibrosis) - a progressive inflammatory lung lesion of unknown nature, which leads to the development of diffuse fibrosis, chronic respiratory and cardiopulmonary failure.

Hamman-Rich syndrome is a rare disease characterized by rapidly progressive diffuse pulmonary fibrosis with the development of respiratory failure, pulmonary hypertension and cor pulmonale.

                  

 Etiology. Hereditary disease with autosomal dominant type of inheritance.

Pathogenesis. Previously in the development of syndrome there was attached importance of auto-sensitization in connection with which diseases was entered to a group of connective tissue disease. Now there is considered that the syndrome occurs as the result of repeated inflammatory bronchopulmonary diseases. Morphological substrate is perialveolar fibrosis, which reduces the elasticity and pliability of lung tissue and thus worsens the excursion of the lungs. Due to the thickening of the interalveolar septa diffusion of gases in the blood is damaged, which leads to hypoxemia and hypoxia, hypercapnia.

                                  Clinic

                    The disease manifests itself mainly in school age children and adolescents, but the first signs of it (on history) almost half of the patients are detected in pre-school age. The disease begins gradually after an attack of influenza, repeated pneumonia, bronchitis and often measles. There are a spastic, dry cough, shortness of breath and cyanosis during physical exertion, sometimes chest pain, feeling of tightness in the chest.

                    Percussion defines an insignificant shortening of sound in the basal zones or no change, decrease of excursion of lungs, reducing difference of lung volume during inspiration and expiration. Auscultation reveals inconstant fine bubbling or crepitation moist rales, sometimes may be weakening of breathing in the lower areas.

                   Further there are increasing of cyanosis around the mouth, acrocyanosis, fingers take the form "drumsticks". The child retards in weight and growth. Thorax is flattened, circumference of the neck disproportionately increases (due to increased contractility of the neck muscles that perform the role of auxiliary respiratory muscles during difficult breathing). Pulmonary heart syndrome is developing with the expansion of the cardiac dullness, signs of cardiovascular failure.

              In the blood – polycythemia, may be polyglobulinemia, ESR is increased, especially in acute periods, concentrations of gamma-globulin is elevated.

             The study of respiratory function reveal the decline of lung volumes, reduced lung compliance and difficulty in passing the oxygen through the alveolar-capillary membrane, and therefore change in the gas composition of the blood (hypoxemia, then hypercapnia) and indicators of acid-base status.

                     

                    After treatment with corticosteroids LVC, total lung capacity, the inspiratory volume is practically unchanged. Radiological investigation in the initial period reveals that the interstitial pattern is enhanced, later nodular formation appear on its background. In periods of exacerbations there is determined multiple shadowing, alternating with areas of particularly clear lung fields. Shadowing is usually localized in the roots and lower parts. Pneumothorax may be possible complication.

                                                                     

                                                   Representative CT scan image from a person with

                                                        idiopathic pulmonary fibrosis.

                                                                             

                                                                    Idiopathic fibrosing alveolitis.

Detail of chest radiograph with                                                                          interstitial pneumosclerosis with a primary lesion of the alveolar connective tissue (fibrosing alveolitis):  small meshy pattern of lung.

Micropreparations of lung at diffuse interstitial pneumosclerosis: interseptums are extremely thickened and sclerotic; coloured by Van Gieson; × 24.

A. Lung showing extensive interstitial and alveolar fibrosis.

    Note the increased interstitial cellularity with numerous fibroblasts.

B. Patchy areas of alveolar septal thickening and prominent hyaline membranes.

C. Higher magnification showing typical dense laminated hyaline membranes.

D. Alveolar septum showing prominent type II pneumocyte proliferation.

E. Abundant polymorphonuclear leukocytes fill alveolar spaces with focal destruction of alveolar septa.

F. Higher power magnification showing the antraalveolar exudate

composed mainly of polymorphonuclear leukocytes, red blood cells, and fibrin.

Original magnifications: A & B, x 50; C, x 100; D, x 158; E, x 50; F, x 158.

Electron-microscopic picture of fibrosed alveoli.

                    CT-image of meshy lung

Vacuolization of macrophages in amiodarone alveolitis. Materials obtained through bronchoalveolar lavage.

            The disease is undulating with periods of exacerbation and remission. However, remission time zone fibrotic changes do not disappear, and rates of respiratory still significantly reduced. Depending on the frequency and duration of periods of exacerbation, some authors distinguish subacute and chronic forms of course. In subacute form periods of exacerbation are more frequent, accompanied by fever reaction, while at chronic progression of pulmonary fibrosis occurs gradually and manifests the growth of respiratory failure. At present, long-term treatment of patients with corticosteroid differences within these two forms have become less pronounced. There are two clinical forms of idiopathic diffuse pulmonary fibrosis depending on diffusion of gases in the lungs. In most cases diffusion capacity is decreased, but in 10-15% of patients it is within normal limits. In such patients the process is easier, retarded growth and weight are insignificant or absent.

               Diagnosis is mainly based on typical clinical signs of disease.

                  Diagnostic criteria of IFA

Clinical

·                               progressive dyspnea

·                               cyanosis

·                               dry cough

·                               progressive loss of weight

·                               tender crepitation wheezing

·                               thickening of hand phalanges nails

Radiographic

·                               diffuse fibrosis

·                               deformation of the pulmonary pattern

·                               presence of focal shadows

Indicators of external respiration

·                               decrease of lung volumes

·                               hypoxemia

Indicators of regional lung function

·                               diffuse irregular distribution of radionucleids

·                               regional lung volume reduction

·                               smoothing apex-basal ingredients

.

                       Differential diagnose.

                 Differential diagnosis should be carried out with exogenous allergic alveolitis caused by the inhalation of various organic antigens. Such condition is observed in children with close and prolonged contact with animals and birds. Clinically, the disease is very similar to idiopathic diffuse pulmonary fibrosis. Exogenous allergic alveolitis is diagnosed based on the detection of precipital antibodies against animal serum and excrements. Excluding the effects of antigen in combination with corticosteroid therapy in such patients may lead to recovery. In idiopathic pulmonary fibrosis diffused all lung volumes, lung elasticity during therapy with corticosteroids did not decreased even at satisfactory state of health of patients and the absence of exacerbations process.

                                           Treatment.

             Corticosteroid therapy is used. Originally prednisolone is prescribed at a dose of 1 mg / kg per day. The dose is gradually reducing, but maintenance therapy (5-10 mg prednisolone per day), continue for many months. In severe progressive course corticosteroids are combined with cytostatic drugs (azathioprine, etc.). During exacerbations, the accession of pneumonia, bronchitis, corticosteroids are combined with antibiotics. In addition, antihistamines, vitamins, symptomatic treatment is used.

                             Prognosis is unfavorable.

ACUTE BRONCHIOLITIS

Development of total obstruction of respiratory tracts lies in basis of syndrome, which is  conditioned with the expressed hypersecretion of phlegma, with the edema and swelling of mucus of  bronchial tubes and bronchiols and, in a less measure, bronchospasm in  the children of the first year of life on a background of viral, more frequent respiratory-syncitial infections.

Criteria of diagnosis of bronchiolitis:

1. Presence of the catarrhal phenomena (rhynopharyngitis, cough).

2. Temperature of body – subfebrile, rarely 38^oС.

3. Expressed signs of respiratory insufficiency:  shortness of breathing, blowing nostrils, cyanosis of nasolabial triangle, participation of auxiliary musculature.

4. Violation of the bronchial passage: thorax is emphysematosis; horizontal position of ribs, flat position of diaphragm.

5.Bandbox sound at percussion.

6. Auscultation: hard  breathing  with prolonged inspiration, moist wheezes, on outbreathing - dry, whistling.

7. Cardiovascular syndrome – tachycardia, weakening of tones.

8. X-ray- sharp swelling of lungs tissue, increased broncho-vascular pattern, without infiltrative shades; sometimes atelectasis.

Clinic. Syndrome of airway obstruction lies at the basis of acute bronchiolitis, which is more typical for children of the first six months of life. In acute bronchiolitis body temperature is subfebrile or normal, respiratory failure progresses gradually. Signs of intoxication are insignificant. Characteristic features are perioral or acrocyanosis, dry cough, a large number of small moist rales on both sides - "wet" lung and a small number of dry wheezes.  

In severe cases dyspnea has predominantly expiratory character, groaning breath, tension and swelling of nostrils, participation in the act of breathing supporting muscles, general cyanosis of the skin. Nonproductive cough, frequently emphysematous swelling of the chest are present. Above the lungs percussion sounds are bandbox, throughout the lungs a large number of small bubbling and wet crepitative wheezing. There are tachycardia, increased liver,  may be attacks of apnea, hypoxic seizures, dehydration.

X-ray examination shows the symmetric strengthening of pulmonary pattern of  both sides, increase of transparency of pulmonary tissue, absence of infiltratative shades. In the blood test the appropriate changes are absent.

Help on prehospital stage.

1.             To release from clothes.

2.             To give the promoted position of body to the child.

3.             By a rubber bulb or gauze clean the  nasal cavity, mouth and pharynx

                     from mucus and phlegma.

4.             To provide access of fresh air.

5.             Oxygenation with clean moistened oxygen through a mask.

6.             Euphyllini in dose of 3-5 mg/kg of mass -24 % solution intramuscular.

7.             3 % solution of Prednisoloni 1-2 mg/kg of  the masses  for 

                     dose intramuscular

8.             Salt-alkaline inhalation.

9.             Urgent hospitalization.

Help on  hospital stage.

1.             To release respiratory tracts from mucus and phlegma with the help

                       of electrosuccer.

                            

2.             Oxygentherapy with  moistened warm 40 %  oxygen through a nasal

                    catheter or mask 3-5 litres per 1 minute.

3.             Inhalation of broncholytic mixture: Euphillini – 0,3, ephedrine – 0,2,

                     novocaine – 0,25, water – 50,0 ml. On inhalation 3-5 ml, before

                    inhalation to add 1,0 ml of  5 % solution of sodium ascorbinati.

4.             Inhalations of mucolytic drugs – 2 % solution of sodium hydrocarbonatis

                     3-5 ml, Acetylcysteini, Bisolvoni, Lasolvani  2-3 ml on inhalation with

                    next aspiration of phlegma.

5.             2,4 % solution of Euphyllin 3-5 mg/kg of mass on 10 % glucose solution

                      10 ml/kg of mass of intravenously slowly.

6.             3 % solution of Prednisoloni 1-2 mg/kg of the masses, one dose

                        intravenously streamly on 10 %  glucose solution 10 ml.

7.             5 % solution of sodium ascorbinati 0,2 ml/kg of the masses

                    of intravenously streamly on 5 ml of  10 % glucose solution .

8.             Cocarboxylazae 5-8 mg/kg of  mass of intravenously streamly

                     on 5 ml of  10 % glucose solution .

9.             Alkaline drink: 1 % solution of sodium hydrocarbonatis  10-15 ml/kg

                     of mass per day.

10.        Vibration massage of thorax in drainage position.

11.        In default of effect and enlargement of respiratory insufficiency – 

                     clearing of bronchial tree by bronchoscope,  artificial ventilation.

                 

                          Sonotherapy at bronchiolitis

ACUTE OBSTRUCTIVE BRONCHITIS

In the basis of syndrome is violation of the bronchial passage, conditioned by the diffuse inflammatory edema of mucus, hypersecretion and accumulation of mucous and purulent phegma in bronchial tubes, transitory reflex spasm of smooth musculature.

                 Until the 70's, the last century, the term "obstructive bronchitis and its analogs (asthmatic bronchitis, spastic bronchitis, etc.) were widely used by pediatricians. However, in the early 70's a series of epidemiological studies were performed, which showed that distinguish obstructive bronchitis and asthma in the general pediatric practice is almost impossible. Thus, equal sign between these states was put. This approach in older children has largely justified itself, as would save many patients from antibiotic therapy. But in younger children the problem is more complicated. It was established that cromoglycate in these patients is not effective enough or not effective at all. Inhaled bronchodilators such as salbutamol, are ineffective enough or not effective at all at wheezing in children of the first three years of life. Information regarding the effectiveness of inhaled steroids for acute or chronic bronchiolitis was contradictory.

               In addition, numerous studies in our country and abroad have shown that the outcomes of obstructive bronchitis in general are favourable. 54% of children with repeated episodes of obstructive bronchitis stopped hurting after four years, and another 37% of - at a later age, thus recovering is more than 90% of patients. In addition, the presence of obstructive syndrome in the first three years of life can not be considered as a factor predisposing to the appearance of asthma in the future. That is, it became clear that the mechanism of development of wheezing in infants, other than for the older children, and the main role is played not hyper reactivity in bronchial mucosa and muscle spasm and tone of the bronchial wall and edema of bronchial mucosa. This reflects the fact that "wheezing" in young children is a consequence of various causes, including abnormalities of the respiratory system and inflammatory processes of other etiologies.

Clinics: subfebril temperature, trouble, crabbiness of child, cyanosis of lips, nasolips triangle, acrocyanosis. Dyspnea, noisy, with the prolonged inspiration and  distance wheezes. Downing in of interrib intervals, supraclavia areas, jugular pit.  Unproductive coughing. A thorax is emphysematous, at percussion above lungs -box sound, аt auscultation- breathing is hard, with the prolonged inspiration and dry and different moist wheezes, character of which is changed after a cough. Таchycardia. A liver is often enlarged. In the general blood analysis there is not substantial changes or insignificant neutrophyls leucocytosis.

                

                                           Barrel thorax

Diagnostic criteria of acute obstructive bronchitis in children

  - Long whistling breath, which is audible at a distance                         

- Inflated thorax when viewed from (horizontal placement

    ribs) participated in the act of breathing support muscles,

   retraction of the intercostal spaces, signs of respiratory

    failure

- Dry cough, paroxysmal, prolonged and at the end of the first

   week passes in the moist

- Percussion determined bandbox pulmonary sound

- Auscultation: hard breathing, exhaling is prolonged,

   large amount of dry whistling rales. There may be coarse

   bubbling low sound rales

- On the chest radiograph is observed decreased lung

   pattern in the lateral regions of the lungs, and increased in the medial

  (hidden emphysema).

On the X-ray of thorax organs - the strengthening of pulmonary pattern, areas of promoted pneumatization without of infiltration changes in lungs.

     

Radiographs of the chest in front projection with AOB: total marked bilateral increase the transparency of lung fields, depletion of vascular pattern on the periphery with its increasing in the central parts, the expansion of the roots of the lungs and flattening of the diaphragm.

              Differential diagnosis of obstructive bronchitis and pneumonia

Symptoms	Obstructive bronchitis	Pneumonia
Temperature	Subfebril	Hyperthermia
Intoxication	Absent or slight	Expressed
Dyspnea	+	+
Dry whistling rales	+	–
Local moist rales	–	+
Percussion sound	Bandbox	Dull
X-ray	The strengthening of pulmonary pattern	Local (segmental, lobe, focal) infiltrates

    Differential diagnosis of bronchial asthma and obstructive bronchitis  

Symptoms	Bronchial asthma	Obstructive bronchitis
Allergologic anamnesis	Positive	Sometimes positive
Start of illness Catarrhal sings of ARVI	Attack of dyspnea with or without ARVI	Gradual increasing of symptoms
Temperature	Normal, sometimes increased	Everytimes increased
Course of disease	Repeating attack of dyspnea	Frequency of obstructive episodes decreases, recovery is possible
Ig E	Increased	Normal
Eosynophylia in blood	Present	Absent

Help on prehospital stage.

1.             To provide access of fresh air.

2.             To release from squeezing clothes.

3.             Succing with electrosuccer mucus and phlegma from upper

                     respiratory tracts.

4.             Oxygentherapy with  clean moistened oxygen through a mask.

5.             Broncholytin for  children upto 3 years– harf of  tea-spoon, 3-10 years

                    a 1 tea-spoon, more senior than 10 years  a 1 dessert-spoon 3 times per

                   day or Solutani 5-10 drops 3 times per  day.                                                                                                                                                     

6.             Euphyllini in  dose of a 3-5 mg per kg of  mass orally or 24 % Euphyllini

                             solution intramuscular.                                   

7.             Salt-alkaline inhalation.

8.             In default of effect - hospitalization.

Help on  hospital stage.

            1. Succing with electrosuccer mucus and phlegma from upper respiratory

               tracts.

            2. Oxygentherapy through the nasal catheter with 40 % moistened oxygen

               constantly.

               

Nasal catheters for oxygenotherapy

            3. Inhalations of broncholitic mixture: Euphyllini - 0,3, ephedrine  – 0,2,

               novocaine – 0,25, water – 50,0 мл. On inhalation 3–5 ml, before inhalation

               to add 1,0 ml of  5 % sodium ascorbinati solution .

            4. Mucolytic drugs: salt-alkaline inhalations; Acetylcysteini or Lasolvani in

               inhalations, syrup, tablets; Mucaltini in tablets, extract of altey.      

            5. Vibromassage of  thorax in drainage position with next active aspiration

                of phlegma.

           6.  2,4 % solution of Euphyllini 3-5 mg/kg of  mass of intravenously

                 in drops on isotonic solution of chloride sodium  10-15 ml/kg of mass.

          7. 5 % solution of sodium ascorbinati 0,2 mg/kg of the masses intravenously

               streamly on 5 ml of  10 % glucose solution .

          8. Cocarboxylazae 5-8 mg/kg of mass of intravenously streamly on 5 ml of 

              10 % glucose solution.

          9. In default of effect from previous therapy Prednisoloni 1–2 mg/kg of the

              masses (or Hydrocortisoni 5 mg/kg of  mass) intravenously streamly on

             5 ml of  10 % glucose solution.

          10. Alkaline drink: 1 % solution of sodium bicarbonates 10-15 ml/kg of