XAMMEHA — RYChA the SYNDROME
(L. V. Hamman, an amer. doctor, 1877 — 1946; A. R. Rich, amer. doctor, sort. in 1893; synonym: a diffusion idiopathic intersticial pneumosclerosis, a diffusion fibroziruyu-shchy alveolitis, an idiopathic fib-roziruyushchy alveolitis) — quickly progressing diffusion pneumofibrosis with development of respiratory insufficiency, hypertensia of a small circle of blood circulation and a pulmonary heart.
In 1944 Mr. Hammen and Rich described «an acute diffusion intersticial pneumosclerosis» with quickly accruing short wind, cyanosis and formation of a pulmonary heart. In 1964 J. G. Scadding for designation of this disease offered the term «fibroziruyushchy alveolitis» to a large extent opening a being of the peculiar inflammatory process developing in an interstitium of a lung.
The disease meets at various age, a little more often at men.
The etiology is not established. Concept of a virus etiology of X. — The river of page did not find confirmations. Among the possible reasons allocate a number of pharmaceuticals (the geksame-Tonian Period, Myelosanum, nitrofurans, streptocides), professional harm. There are instructions on the hereditary nature of the disease which sometimes is found in several generations of one family.
Pathogeny. Pathogenetic mechanisms of development of X. — Rubles of page are not quite clear. A combination fibro-ziruyushchego an alveolitis with general diseases of connecting fabric, first of all with a pseudorheumatism (see) and a system scleroderma (see), is more rare — with a dermatomyositis (see), Shegren's syndrome (see Shegren a syndrome), a system lupus erythematosus (see), and also hron. active hepatitis confirms an important role immunol. disturbances in a pathogeny of this disease. As confirmation to it serves also frequent identification in blood of patients with X. — River of page of rhematoid and antinuclear factors, to a hyper-gammag lob of a linemiya, I circulate
shchy cell-bound immune complexes, and in an interstitium of lungs — gistiolimfo-cytic infiltration. Immunol. reactions, in to-rykh take part cell-bound immune complexes, system of a complement, alveolar macrophages with activation of a neutrophylic chemotaxis, lead to accumulation in alveolar structures of neutrophils. At death of the last a large amount of proteolytic enzymes, including collagenases (see) is released that conducts to qualitative and, perhaps, to quantitative changes of collagen in an interstitium of lungs and to development of an alveolitis (see Pneumonia). These changes, according to R. G. Crystal with sotr. (1980), are also main reason for bystry progressing of fibrosis. Formation of a so-called neutrophylic alveolitis is confirmed by data of studying of cellular composition of the liquid received at bronchoalveolar lavage (washing) and in material of a «open» biopsy.
Pathological anatomy. Pathological changes in lungs at X. — Rubles of page are characterized by development in alveolar partitions of alterativno-pro-duktivnoy reaction. Note the accruing diffusion proliferation of stromal cells of lungs, cells of an alveolar vystilka, a necrosis of an alveolar and bronchial epithelium, formation of hyaline membranes, swelled.
Allocate two main a patomor-foul. option X. — River of page: desquamative, for to-rogo existence in gleams of alveoluses of alveolar pnevmotsit of the II type and the moderate phenomena of fibrosis (so-called desquamative pneumonia), and ste-nochny (muralny) — with dominance of diffusion fibrosis is characteristic. It is impossible to exclude that these options are stages of uniform process. In process of progressing of fibrosis the obliteration and deformation of alveoluses is observed. At the same time respiratory bronchioles also are involved in process. Further as a result of a rupture of walls of alveoluses and merge of alveoluses and bronchioles there is a formation of small smooth-bore cavities — a so-called cellular lung.
To l and N and h e with to and I cards and -
II and. The disease sometimes can sharply begin, reminding an acute respiratory disease with fever, cough with a phlegm and quickly accruing asthma. In other cases the symptomatology develops gradually. At an objective research widespread ringing crepitation in lower parts of lungs comes to light. Conducting a wedge, signs are: I accrue
a shchy asthma, unproductive cough, feeling of constraint in a breast. Eventually the phenomena of respiratory insufficiency accrue (see), cyanosis (see), symptoms of hypertensia of a small circle of blood circulation (see) and hro appear. pulmonary’ hearts (see). The thickening of trailer phalanxes in the form of «drum sticks» is quite often noted (see. Drum fingers). In some cases the pneumorrhagia is observed (see). Emergence of fever at a certain stage of a disease in some cases svyazash> with emergence of complications, napr, bacterial pneumonia.
The diagnosis establish on the basis of characteristic a wedge, signs. Among additional methods of a research is important radiological. Early rentgenol. signs of X. — Rubles of page are strengthening and deformation of the pulmonary drawing which are usually revealed in the lower and average parts of pulmonary fields. Transparency of these sites goes down due to emergence of a set of the tyazhisty shadows going radially from roots to the periphery on the course of bronchial tubes and vessels. The mesh, cellular drawing caused by growth of connecting fabric around segments and an acinus (fig.) is characteristic.
In process of progressing of process consolidation of shadows of roots and a thickening of a pristenochny, interlobar and diaphragmal pleura is marked; upper parts of lungs are also gradually involved in patol. process. Against the background of the strengthened and deformed pulmonary drawing there are indistinctly outlined focal shadows of the small and average sizes which are partially merging among themselves. Mobility of a diaphragm decreases, costal and phrenic sine partially, and then are completely obliterated.
At far come process in a cortical layer of lungs on the periphery of pulmonary zero
Fig. appear. The roentgenogram of a thorax of the patient with a syndrome Hammen — Rich (a direct projection): sharp strengthening and tyazhi-ssto-cellular deformation of the pulmonary drawing.
gkistovidny cavities, with a diameter up to 2 — 3 cm, nek-ry with a fluid level. A tomography (I eat.) and a bronchography (I eat.) allow to find sometimes the cylindrical, meshotchaty and mixed bronchiectasias, usually in nizhnemedialny departments of lungs. Characteristic of a current of X. — The river of page is steady increase noted rentgenol. changes.
The research of function of external respiration reveals restrictive type of disturbance of ventilation (see. Respiratory insufficiency), decrease in diffusion capacity of lungs, existence of an arterial gi-ioksemiya (see the Hypoxia). In a pathogeny of an anoxemia at X. — Rubles of page along with development of the alveolar and capillary block are important disturbances of the ventilyatsion-but-perfused relations.
For specification of the diagnosis in some cases carrying out transbronchial is reasonable (see Bronkhoskopiya, t. 15. additional materials) or «open» transthoracic biopsy of a lung. For the purpose of specification of activity of process conduct a research of the liquid received at bronchoalveolar lavage.
In blood quite often note acceleration ROE, a respiratory hyperglobulia, a hypergammaglobulinemia, existence of rhematoid and antinuclear factors, increase in level • separate classes of immunoglobulins.
D Ife r an entsi to flax yu diagnosis is carried out with a pulmonary tuberculosis (see Tuberculosis of a respiratory organs),
■ a sarcoidosis (see), a pneumoconiosis (see), pneumomycoses (see), a lymphogenous carcinomatosis, other pneumoscleroses (see the Pneumosclerosis).
JI e h e N and e. The main pharmaceuticals used in an initial phase of a disease, yav by l I to Utah I am of l yuko to r tikos those r about ides.
Most often appoint Prednisolonum. In the presence of the expressed intersticial pneumosclerosis apply cytostatics (Azathioprinum, cyclophosphamide), and also cuprenil.
Forecast most often adverse. Average life expectancy from the moment of emergence of the first symptoms of 4 — 6 years.
However at timely begun and purposeful treatment quite often it is possible to prolong life of patients for many years and even to keep their working capacity.
Bibliography: Clinical X-ray radiology, under the editorship of G. A. Zedgenidze, t. 1, page 145, M., 1983; Rozenshtra-
at x L. S., Rybakov N. I. and M. G Vanner. Radiodiagnosis of diseases of a respiratory organs, page 463, M., 1978; T a p e e in E. M and Nasonova V. A. Nonspecific pulmonary reactions in therapeutic clinic, in book: Acute pneumonias, under the editorship of V. of X. Vasilenko and G. V. Vygodchikov, page 47, M.,
1961; T e r N of e r - At about r in and to M. Immunologiya of lungs, the lane with English, M., 1982; Crystal R. G. and. lake of Interstitial lung disease, Amer. J. Med., v. 70, p. 542, 1980; Fishman A. P. Pulmonary diseases and disorders, N. Y. and. lake, 1980; H a m m a n L. Heart pain of organic origin, Int. Clin., y. 2, p. 157, 1935; H a m-man L. Rich A. R. Acute diffuse interstitial fibrosis of the lungs, Bull. Johns Hopk. Hosp., v. 74, p. 177, 1944; Reynolds H. Y. Immunologic lung diseases, Chest, v. 81, p. 745, 1982; Sea d-d i n g J. G. Diffuse pulmonary alveolar fibrosis, Thorax, v. 29, p. 271, 1974;
Spencer H. Pathology of the lung, p. 728, Oxford a. o., 1977;
Turner-Warwick M., Burrows B. Johnson A. Cryptogenic fibrosing alveolitis, clinical feature and their influence on survival, Thorax, v. 35, p. 171, 1980.
B. M. Kornev; JI. S. Rozenshtraukh (rents.).