Hammenarich Syndrome (Idiopathic Pulmonary Fibrosis)

Hammenarich syndrome (idiopathic pulmonary fibrosis) is a rare disease characterized by exclusively pulmonary localization of the process, little effect of therapy and frequent death. Despite the rarity of the disease, its frequency is increasing, and patients need a quick and accurate diagnosis, as well as comprehensive treatment.

The etiology of Hammenarich syndrome is unclear, but the underlying pathogenesis of the disease is believed to be autoimmune. The main pathomorphological substrate is the alveolar-capillary block, which leads to impaired diffusion function of the lungs. Characteristic features include serous-fibrinous impregnation of the alveolar septa, accumulation of exudate rich in fibrin and macrophages in the alveoli, pronounced proliferation of connective tissue and its sclerosis, hyaline membranes in the alveoli. The lungs are dense, red-brown in color with a network of grayish stripes and foci of bullous emphysema.

Hammenarich syndrome is characterized by severe, progressive and treatment-resistant symptoms such as shortness of breath and cyanosis. Hypoxemia increases, pulmonary hypertension and right ventricular failure develop. Cough, “drum fingers”, bilateral crackling crepitant rales, dry pleurisy are noted; Spontaneous pneumothorax is possible. Characterized by a restrictive type of ventilation impairment and a progressive decrease in the diffusion capacity of the lungs.

To diagnose Hammenarich syndrome, X-ray methods and changes in leukogram, proteinogram, ESR and increased body temperature, usually caused by a secondary infection, are used.

In the early stages of the disease, prednisolone is prescribed up to 40 mg/day with a gradual reduction in dose when a clinical effect is achieved. In the late period of the disease and with a rapidly progressing course, glucocorticoids are combined with immunosuppressants (azathioprine 2.5 mg/kg per day for 2 months, then 1.5 mg/kg per day for 1 year or more) and penicillamine or cuprenil ( 300 mg/day, then gradually increase the dose to 1.8 g/day, followed by a decrease to maintenance - 300 mg/day). At the same time, potassium preparations, veroshpiron and pyridoxine are used.

However, despite treatment, the prognosis of Hammenarich syndrome remains poor, and most patients die within a few years of diagnosis. Therefore, the main goal in the treatment of Hammenarich syndrome is to improve the quality of life of patients and prevent the rapid progression of the disease.

In conclusion, Hammenarich syndrome (idiopathic pulmonary fibrosis) is a rare and severe disease characterized by progressive and treatment-resistant symptoms and frequent mortality. Although the etiology of the disease is unclear, comprehensive treatment including glucocorticoids and immunosuppressants can improve patients' quality of life and slow disease progression. However, the prognosis remains poor and most patients die within a few years of diagnosis.