The primary immunodeficiencies are the cause of recurrent infections of respiratory system. Sometimes they develop into severe irreversible pulmonary involvement with the development of chronic respiratory failure. Job’s syndrome (also called Hyper-IgE syndrome, HIES) is a rare type of primary humoral immunodeficiency. Recurrent pneumonia, mostly caused by Staphylococcus aureus, Streptococcus pneumoniae and Haemophilus influenzae, is typical for this disease. Multiple pneumatoceles of lung (gas-filled cavities) occur later. Superinfection caused by Pseudomonas aeruginosa and mycotic pathogens – Aspergillus, Scedosporium is possible. Complications, like hemoptysis, empyema, pneumothorax and metastatic brain abscesses, worsen the prognosis. Bronchiectases and lung fibrosis commonly develop as well as respiratory insufficiency in the terminal stage. Standard treatment of Job’s syndrome currently does not exist. Prognosis of this disease is unclear. We report a patient with Job’s syndrome accompanied by serious pulmonary involvement. Left side pneumonectomy was performed early in the childhood due to chronic infection. Bronchiectases, pneumatocele and incipient interstitial lung fibrosis of right lung appeared later. These advanced changes were complicated by Aspergillus flavus superinfection, recurrent massive hemoptysis and later by empyema.

Key words: primary immunodeficiency, Job’s syndrome, Hyper-IgE syndrome, HIES, pulmonary involvement

Pulmonary hyalinizing granuloma (PHG) is a rare condition of unknown etiology. It is commonly characterized by solitary or multiple nodular lesions in pulmonary parenchyma. The association with autoimmunity signs is common. The differential diagnosis is wide-ranging. The course of the disease is mostly benign and does not require any treatment. Nevertheless, the progression of the disease causing clinically significant problems is seen in about one-third of patients. We present a patient with histologically proved PHG and autoimmune symptoms. Complete regression of pulmonary involvement and accompanying hematological abnormities was achieved by combined immunosuppressive therapy.

Key words: pulmonary hyalinizing granuloma, PHG, management

The case history describes a patient born in 1946. He was diagnosed with miliary pulmonary tuberculosis in 1979 that was cured by combined chemotherapy. Later on, the patient was diagnosed with two types of pulmonary mycobacteriosis: mycobacteriosis caused by M. kansasii in 1993 and mycobacteriosis caused by M. xenopi in 2005; both infections were cured by combined chemotherapy. Clinically non-significant species M. fortuitum and M. nonchromogenicum respectively, were diagnosed in patient’s sputum in 2002 and 2003; and M. xenopi again in 2007. The patient also suffered from recurrent attacks of nonspecific inflammatory affections of respiratory tract for many years. Non-tuberculous mycobacteria infections and recurrent nonspecific infections developed in noncompliant patient who had pulmonary involvement caused by cured tuberculosis that was followed by chronic obstructive pulmonary disease and pulmonary emphysema and potentiated by long term smoking and alcohol abuse.

Key words: tuberculosis, mycobacteriosis, M. kansasii, M. xenopi, COPD