Intra-Alveolar Hemorrhage in Certain Orphan Lung Diseases in Children (Lecture)

The theme is relevant due to the diagnostic difficulties, severe clinical course and prognosis of idiopathic pulmonary hemosiderosis and Goodpasture syndrome. Idiopathic pulmonary hemosiderosis and Goodpasture syndrome are severe, life-threatening immunopathologic diseases due to alveolar hemorrhage and a hundred percent mortality within a short period of time after the onset of clinical manifestations. Idiopathic pulmonary hemosiderosis generally occurs in children at the age of 3–8 years as a separate condition, or as a stage of Goodpasture syndrome. It manifests itself as shortness of breath, pneumonia, prune juice sputum, hemoptysis, hemorrhage. During exacerbation, the patients' condition is determined by the degree of pulmonary hemorrhage, pulmonary heart disease, acute posthemorrhagic anemia. The exacerbation lasts from a few hours to 1–2 weeks. The duration of each episode and remission varies among patients being unpredictable. Each new exacerbation is more severe. In Goodpasture syndrome, predominant pulmonary and renal vascular lesions of autoimmune nature are observed. It affects young males more frequently; is rare in children. Hemorrhagic alveolitis as a form of lung damage develops first; then, the kidneys are involved, and anemia occurs. Glomerulonephritis (GN) manifests itself as nephrotic syndrome with rapid progression of kidney failure. In case of the predominant pulmonary pathology, recurrent hemoptysis and pulmonary hemorrhage are observed; in end-stage disease with cardiopulmonary failure manifestations, rapidly progressive GN and kidney failure develop. The second variant of Goodpasture syndrome is characterized by relatively slow progression of pulmonary changes and renal lesions. Goodpasture syndrome is rarely accompanied by GN from the onset to the end of the disease and pulmonary pathology manifests itself at the terminal phase of the disease. The treatment of both diseases includes lifetime therapy with glucocorticoids, cytostatics. Pulse therapy using these preparations, discrete plasma exchange and intravenous immunoglobulin administration, syndromic treatment are carried out. No conflict of interest was declared by the authors. Keywords: children, idiopathic pulmonary hemosiderosis, Goodpasture syndrome, clinical course, therapy.

Goodpasture syndrome as a rare cause of massive respiratory tract hemorrhage

In this study, the cases of a patient with Goodpasture syndrome (also referred to as anti-glomerular basement membrane disease) and a patient with the morphological features of Goodpasture syndrome or Goodpasture-like syndrome found in a histopathological examination are described. In both cases the course of the disease was atypical, with a massive respiratory tract hemorrhage being the most significant symptom. Accordingly, the presented patients were treated surgically. Furthermore, the paper described diagnostic difficulties in identifying the cause of the hemorrhage. Finally, the importance of histopathological examination in the diagnosis of Goodpasture syndrome and the Goodpasture-like syndrome with atypical course is emphasized.