[Pulmonary manifestations of polymyositis and dermatomyositis: a report of 6 cases and a review of literature]. / Zmiany plucne u chorych na zapalenie wielomiesniowe lub skórno-miesniowe: opis 6 przypadków i przeglad pismiennictwa.

The group of 6 patients with interstitial pulmonary changes in the course of polymyositis or dermatomyositis treated in years 1986-1999 was assessed. In one woman pulmonary changes occurred ten years before other symptoms of the connective tissue disease. Lung function tests showed a restrictive ventilatory defect in all patients. High resolution computed tomography revealed different changes, from ground glass attenuation to honey--combing. Only one patient, probably with bronchiolitis obliterans organizing pneumonia, responded to corticosteroids. Four women died from respiratory failure. In 2 pneumomediastinum occurred, which is a rare complication.

[Pulmonary lymphangioleiomyomatosis: presentation and results of treatment]. / Limfangioleiomiomatoza pluc: obraz kliniczny i wyniki leczenia.

Lymphangioleiomyomatosis is a rare lung disease of unknown aetiology that affects only women. Eight premenopausal women with LAM confirmed by lung biopsy specimens were observed in 1984-2001. The most common presenting feature was exertional dyspnea (6) followed by chylous pleural effusions and pneumothoraces. In two women severe airflow obstruction was observed at presentation. HRCT revealed characteristic cysts in all cases. All women were given hormonal therapy (tamoxifen, medroxyprogesterone). The best results of treatment were achieved in cases with chylothoraces.

[Usefulness of analyzing ANCA changes for diagnosis of Wegener's granulomatosis]. / Przydatnosc oznaczania miana ANCA dla diagnostyki ziarniniakowatosci Wegenera.

UNLABELLED: ANCA were described in 1982 as sensitive and specific markers for active Wegener's granulomatosis (WG). We analysed the results of ANCA test performed in 298 patients hospitalized in Institute of Tuberculosis and Lung Diseases in the period 1990-1998 because of different symptoms and syndromes of respiratory system which could be symptoms of WG. Presence of c-ANCA, p-ANCA and other not well defined types of ANCA in the titer greater than 1:40 in the serum was regarded as positive result of test. We found it in 60 patients. In 47 WG pts ANCA were present in 40 of 42 pts with active disease and 1 of 5 in remission. Further 19 positive results were found in a group of 251 patients with other diseases: 1 of 4 pts with Churg-Strauss syndrome, 1 of 2 with pulmonary renal syndrome, 5 of 28 with connective tissue diseases, 1 of 21 with tuberculosis, 1 of 23 with sarcoidosis, 1 of 6 with histiocytosis, 3 of 11 with hypersensitivity pneumonitis, 1 (lymphoma) of 34 with neoplasms, 1 of 20 with pulmonary fibrosis, 1 of 8 with cardiac failure, 1 of 5 with pleural fluid, 1 of 10 pneumoconiosis and toxic reactions (after furagin), 1 of 6 with BOOP. Sensitivity of ANCA test in our material is 87%, specificity = 95%, and positive prevalence accuracy is 68%. It means that 32% of the patients with positive results could be inappropriate treated as WG. CONCLUSION: ANCA test could be not used as a screening test. Results of ANCA test alone cannot be used as basis for treatment. ANCA test is a helpful tool in diagnosing of WG.

Kabuki (Niikawa-Kuroki) syndrome associated with immunodeficiency.

We report a case of a 19-year-old male with the cardinal features of the Kabuki syndrome (KS) and, in addition, with severe immunodeficiency. Finding immune deficiency in a KS patient, prompted us to determine whether this association was related to a deletion within the DiGeorge chromosomal region. Fluorescence in situ hybridization (FISH) with the Oncor probe N25(D22S75) revealed no deletion of 22q11.2 in the patient.

[Common variable immunodeficiency--analysis of the clinical aspects and results of substitution treatment]. / Pospolity zmienny niedobór odpornosci--analiza obrazu klinicznego i wyników leczenia substytucyjnego.

The clinical picture and the effects of treatment with intravenous immunoglobulins of 14 adult patients (4 women, 10 men) with common variable immunodeficiency are presented. In most patients the disease began in the second decade of life (17.4 years on average), and the diagnosis was delayed by 7.5 years. Mean observation time was 38.5 months. Recurrent sinopulmonary infections were the most significant clinical feature and were observed in all patients, although chronic diarrhea and viral hepatitis, predominant of B type, were also frequently seen (both in 57% of patients). The diagnosis was made according to following criteria: frequent bacterial infections of the respiratory tract, serum IgG levels reduced below 300 mg/dl, (mean, 60.7 mg/dl), normal numbers of circulating B lymphocytes and exclusion of the secondary type hypogammaglobulinaemia. After 6 months of treatment with fresh plasma 20 ml/kg or intravenous immunoglobulins 200-400 mg/kg, mean serum IgG levels rose to 654 mg/dl. Treatment was clinically efficacious and markedly reduced the number of infections of the respiratory tract. The delay of the diagnosis, resulted in high rate of irreversible damage of respiratory tract observed in our group of patients.

[Use of methotrexate for treatment of corticosteroid-dependent asthma]. / Zastosowanie metotreksatu w leczeniu kortykozaleznej astmy oskrzelowej.

We evaluated the effects of methotrexate treatment in 20 subjects (10 men, 10 women) suffering from corticosteroid-dependent asthma. All patients had been treated with 15 mg of prednisone or more per day for at least 3 years before including in the study. Methotrexate (15 mg once weekly) had been given for 9.7 months on average. Oral steroid intake was reduced by 50% in 8 patients and it was possible to discontinue oral corticosteroids in further 6 patients without deterioration of spirometric parameters at the end of the study. Side effects of methotrexate therapy were observed in 40% of patients, and required the discontinuation of treatment in 6 subjects.

[Diagnostic difficulties with detection of foreign bodies from the lower respiratory tract--late detection]. / Trudnosci diagnostyczne w rozpoznawaniu cial obcych w dolnych drogach oddechowych--pózne ich wykrywanie.

Five cases of aspirated foreign bodies in bronchial tree were described. The clinical symptoms did not suggest the real cause of the illness and most of patients did not remember the fact of foreign body aspiration. The foreign bodies were removed during bronchofiberoscopy performed in local anaesthesia.

[Pulmonary changes as a result of pulmonary artery involvement during Takayasu disease--case report]. / Zmiany w plucach jako wynik zajecia tetnic plucnych w chorobie Takayasu--opis przypadku.

We described a case of Takayasu disease with pulmonary artery involvement in a young woman with dyspnea, and multiple pulmonary infiltrations on chest radiogram. The changes were initially diagnosed and treated as protracted pneumonia. In the later stage of the disease the development of the typical inflammatory changes in the systemic arteries with absence of the left radial pulse suggested the diagnosis.