Towards the diagnosis of Hammen-Rich syndrome

Hammen-Rich syndrome belongs to the group of primary pulmonary fibrosis. Its etiology is unknown. Morphological changes in this disease are characterized by the development of diffuse inflammatory changes from exudative inflammation to pronounced fibrosis along the course of small vessels, as well as along the interlobular and interalveolar septa. This leads to an alveolar-capillary block with a significant violation of the function of external respiration and progressive shortness of breath. Of all the proposed names of the disease, its morphological essence is most consistent, according to most authors, the definition of "diffuse fibrosing alveolitis". As a syndrome, it is described in such systemic diseases as rheumatoid arthritis, systemic scleroderma, but it is also recognized as an independent nosological form.