[Comparison of HLA class I and DR class II antigen frequency in Polish patients, in relation to different stages of the disease]. / Porównanie czestosci wystepowania swoistosci serologicznych antygenów klasy I i grup alleli DR klasy II ukladu HLA, wsród chorych na sarkoidoze w populacji polskiej, w odniesieniu do stadium choroby.

The aim of this study was to analyze the association between HLA class I and class II DR frequency in the different stages of sarcoidosis in Polish population. 88 patients and 30 healthy controls have been typed. Patients were divided into three groups depending on radiological findings. In the first group were 28 cases presenting the regression of the disease. In the second were 33 patients in stable stage II or III and in the third group 27 patients with pulmonary fibrosis (stage IV). The typing was performed by NIH method using commercial sera. There were no statistically significant differences between studied group in HLA-A class I. The frequency of HLA B-18 was statistically more frequent in patients with sarcoidosis compared to healthy controls. HLA-DR1 was not present in third group of patients and the difference was significant compared to healthy controls.

[Familial sarcoidosis in Poland: analysis of clinical characteristics and environmental aspects]. / Sarkoidoza rodzinna w Polsce: analiza obrazu klinicznego choroby oraz uwarunkowan srodowiskowych.

Sarcoidosis is a systemic disease of undetermined etiology although it can be related to genetic or environmental factors or both. We investigated 19 polish caucasian families with at least two affected relatives and healthy families members in order to evaluate the clinical aspects of familial sarcoidosis and the influence of environmental factors. We have found three types of familial sarcoidosis: 10 sib pairs, 1 sib triplet, 5 parent offspring pairs and one case of sarcoidosis in cousins. Most of sarcoidosis cases were histologically proved. A high frequency of chronic onset of the disease, chronic form with extrapulmonary sarcoidosis and fibrosis were observed. In only three families the course of the disease was similar in both affected relatives. There was no specific environmental agent found that could be related to the development of the disease. We concluded that it may be possible that familial sarcoidosis can have poorer prognosis that non-familial form, but there is no epidemiological data available about the clinical aspects of sarcoidosis in Poland. The clinical aspects of familial sarcoidosis can suggest the inherited susceptibility to the disease. The etiologic extrinsic factor has not been identified, but it doesn't exclude its role in the pathogenesis.

[Evaluation of effects of antituberculous treatment on the course of sarcoidosis]. / Ocena wplywów leczenia przeciwpratkowego na przebieg sarkoidozy.

From 1960 to 1994 2150 sarcoid patients were observed in our Sarcoidosis Clinic. Before the diagnosis of sarcoidosis was confirmed, 52 of those patients were treated with tuberculostatics because of the radiological changes in the lung, diagnosed as tuberculosis. In no case any conventional method-smear examination for acid bacilli or culture identification was positive. There was no radiological improvement after treatment in any patient. Subsequently in all those patients sarcoidosis was diagnosed, by typical histology (48 cases) or on clinical grounds (4 patients). In 15 cases spontaneous remission was observed. 37 patients were treated with steroids and in 31 of them radiological improvement was found. In six cases stabilization of the disease was noted. The observation time after steroids treatment was from one year to 27 years. In only one case tuberculosis of the lung has developed.

[Co-stimulating effect of extracellular matrix proteins on T lymphocyte proliferation in patients with sarcoidosis. Preliminary tests]. / Ko-stymulacyjny efekt bialek ECM na proliferacje limfocytów T u chorych na sarkoidoze. Badania wstepne.

Although very little is known about the aetiology of sarcoidosis, its immunopathogenesis is now better known. The interaction of alveolar macrophages and T cells may play a role in the pathomechanism of the disease. To infiltrate the tissue, lymphocytes have to migrate through the subendothelial basement membrane and interstitium, rich in extracellular matrix (ECM). The interaction of lymphocytes with proteins of the ECM may play an important role in the migration, accumulation and activation of these cells. The aim of our study was to estimate the ability of the ECM components (collagen I, collagen IV and fibronectin) to co-stimulate T-cells in patients with sarcoidosis. The peripheral blood was obtained from 14 sarcoid patients. The disease was confirmed histologically in 9 cases and in 5 patients on clinical grounds. In radiological findings 4 persons were at the first stage of the disease, 4 at the second, in three cases interstitial changes were found and in three patients, the fibrosis on the X-ray was noticed. No one of those patients were treated with steroids during last 2 months. Normal peripheral blood T cells are strongly co-stimulated by ECM proteins. In contrast, lymphocytes from patients with sarcoidosis were inhibited by the ECM proteins. The mean co-stimulation ratios (OKT3 + ECM proteins:OKT3 alone) were significantly lower for all ECM proteins (collagen I: p < 0.00009; collagen IV: p < 0.02; fibronectin: p < 0.04). Our data shed a new light on the nature of sarcoidosis associated immunodeficiency and suggest that disturbed T cells: ECM interactions may play a role in the pathogenesis.

[Recurrence of pulmonary sarcoidosis]. / Nawroty sarkoidozy pluc.

From September 1960 to December 1992, 2021 patients with pulmonary sarcoidosis were registered in our Sarcoidosis Clinic. 462 patients were treated with steroids. Treatment was initiated either because of radiological evidence of disease progression after 6 months observation, lack of radiological improvement together with abnormalities of pulmonary function, presence of extra-pulmonary lesions. In 42 cases (9% of treated group); 19 men and 23 women, mean age 35 years, relapse of disease was observed from 6 months to 6 years after completion of treatment. At the entry to the first treatment 11 patients were assessed as having stage I disease, 30 as stage II and one as stage III. In 16 subjects of this group (38%), extra-pulmonary lesions were presents; skin lesions in 8 cases and enlarged peripheral lymph nodes in 6. After initial treatment, radiological and clinical improvement was observed in 32 patients. The second treatment results in radiological improvement in 38 patients, 10 are being still treated. In 4 cases a second relapse was noted. In three patients regression of pulmonary changes was achieved, in one case stabilization of the disease was observed.

Inhaled budesonide vs prednisone in the maintenance treatment of pulmonary sarcoidosis.

The aim of this investigation was to compare inhaled budesonide with oral prednisone in maintenance treatment of pulmonary sarcoidosis. A double-blind controlled study was performed in 40 patients with stage II or III pulmonary sarcoidosis. After initial systemic 6 weeks treatment with prednisone (40 reduced to 20 mg daily) patients were allocated either to the systematically (S) or topically (T) treated group. S patients continued with 10 mg prednisone daily, T patients were given inhaled budesonide 1.6 mg daily. The progress of treatment was assessed by serial radiography, spirometry, serum Angiotensin Converting Enzyme (ACE) activity and plasma cortisol levels. All patients completed the 12 months treatment. Using the numerical score to assess changes on the chest radiograms S patients improved by 1.7 +/- 0.66 points; T patients improved by 1.15 +/- 0.81 points. Spirometric changes were not statistically significant. Serum ACE fell from 107 +/- 51 U/L in the S group and 92 +/- 40 U/L in the T group to 46 +/- 11 U/L and 38 +/- 21 U/L respectively during the initial phase of treatment. In the maintenance phase ACE levels remained lower than the initial ones in both groups. Morning plasma cortisol levels studied in 10 patients (5 in each group) decreased significantly during the initial phase. Thereafter cortisol levels remained low in the S patients returning to the lower limit of normal values in the T patients. We conclude that inhaled budesonide may be a safe and effective alternative to oral steroids in the maintenance treatment of pulmonary sarcoidosis especially in the early stage of the disease.

[Comparison of the effectiveness of budesonide and prednisone in the maintenance treatment of pulmonary sarcoidosis]. / Porównanie skutecznosci budezonidu i prednizonu w podtrzymujacym okresie leczenia sarkoidozy pluc.

The aim of this investigation was to compare inhaled budesonide vs oral prednisone in the maintenance phase treatment of pulmonary sarcoidosis. Double-blind controlled study was performed in 40 patients with stage II or III pulmonary sarcoidosis. After initial systemic 6 weeks treatment with prednisone (40 reduced to 20 mg daily) patients were allocated either to systematically (P) or topically (B) treated group. P patients continued with 10 mg prednisone daily, B patients were given inhaled budesonide 1.6 mg daily. The progress of treatment was assessed by serial radiography, spirometry, serum ACE activity and plasma cortisol levels. All patients completed the 12 months treatment. Using a numerical score to assess changes on the chest radiograms P patients improved by 1.7 +/- 0.66 points; B patients improved by 1.15 +/- 0.81 points. Spirometric changes were insignificant. Serum ACE fell from 107 +/- 51 U/L in the P group and 92 +/- 40 U/L in the B group to 46 +/- 11 U/L and 38 +/- 21 U/L respectively during the initial phase of treatment. In the maintenance phase ACE levels remained lower than initial ones in both groups. Morning cortisol plasma levels studied in 10 patients (5 in each group) decreased significantly during the initial phase. Thereafter cortisol levels remained low in the P patients returning to the lower limit of normal values in the B patients. We conclude that inhaled budesonide may be a safe and effective alternative to oral steroids in the maintenance treatment of pulmonary sarcoidosis especially in the early, stage II, disease.

Airways obstruction in patients with sarcoidosis.

We present data concerning the frequency of airways obstruction among 1630 patients with pulmonary sarcoidosis diagnosed and followed up in our centre between 1960 and 1988. Airways obstruction was diagnosed when FEV1% VC was below 70%. There were 93 patients in whom ventilatory impairment of the obstructive type was found (5.7%). Further, we divided them into two groups of-pure obstruction and mixed-when obstruction was accompanied by reduced vital capacity. Possible mechanisms of the obstructive pattern in spirography are discussed.

[Bronchial obstruction in pulmonary sarcoidosis]. / Obturacja oskrzeli u chorych na sarkoidoze pluc.

The authors present the incidence of bronchial obstruction in patients with pulmonary sarcoidosis basing on their own material from the years 1960-1988. Bronchial obstruction was diagnosed when the Tiffeneau index was lower than 70%. In the analyzed group 93 (5.7%) patients were found to meet the criterion for bronchial obstruction. Out of this group two forms were selected those with the pure bronchial obstruction and mixed, i.e. with coexistent restrictive changes. The author discuss the possible mechanism of developing bronchial obstruction in sarcoidosis.

Effects of corticosteroid treatment on pulmonary haemodynamics in patients with sarcoidosis.

Effects of 12 months steroid treatment on pulmonary haemodynamics in 24 patients with pulmonary sarcoidosis, stage II and III, were investigated. All patients had a chest radiograph, lung function tests, and pulmonary haemodynamics (measured at rest and during submaximal exercise) prior to the treatment. Resting pulmonary hypertension was found in 3 patients. In 18 an abnormal increase in pulmonary arterial mean pressure (PAP) on exercise was observed. All investigations were repeated after a year of treatment. In almost all patients (22) a regression of radiological changes in the lungs and improvement of pulmonary function were observed. The changes in pulmonary circulation were less uniform. In only half of the patients studied was regression of the disease seen on chest X-ray and improvement in lung function accompanied by improvement in pulmonary haemodynamics.

[Value of transthoracic needle biopsy of the pleura in the diagnosis of diseases of the respiratory tract]. / Znaczenie transtorakalnej iglowej biopsji oplucnej w rozpoznawaniu chorób ukladu oddechowego.

Pleural biopsy with Abram's needle was made in 160 consecutive patients with undiagnosed pleural effusions. The sampled fluid and pleural bioptates were examined histologically, cytologically and bacteriologically. In 40 cases malignant changes were found, out of 87 with confirmed malignancy. Neoplastic cells were found in 26 patients, ovreall the diagnosis was made in 65% of lung cancer cases. Out of 41 cases of tuberculosis, pleural biopsy provided this diagnosis in 24 patients (59%). Bacteriological examination and pathological of the biopsy specimen allowed to increase the diagnostic field to 63%. Pleural biopsy did not yield any diagnosis in 9 cases of circulatory insufficiency, nor in ten patients with the following diagnoses -- pleuropneumonia, reumathoid arthritis, pulmonary thromboembolism and thoracic trauma. In 13 cases the diagnosis was not made. The diagnostic yield of pleural biopsy was similar to that reported by others. It is most valuable in cases suspected of malignancy and tuberculosis.

[The frequency and course of fibrotic changes in patients with sarcoidosis]. / Czestosc wystepowania i przebieg zmian wlóknistych u chorych na sarkoidoze pluc.

Basing on observations of 960 patients with pulmonary sarcoidosis the authors found fibrotic changes of pulmonary parenchyma in only a few, the incidence being 3.2. Improvement in radiological examination following steroid therapy was seen in 50%, of respiratory function in 30%. Although both examinations do not fully correlate with each other, they are however supplementary with each other, and should be regarded as important criteria of functional involvement and therapy outcome.

[Effect of steroid therapy on pulmonary circulation in pulmonary sarcoidosis]. / Wplyw leczenia kortykosteroidami na krazenie plucne u chorych na sarkoidoze pluc.

Effect of 12 month steroid therapy on pulmonary circulation at rest and during exercise was assessed in 24 patients with histologically confirmed stage II and III pulmonary sarcoidosis. Pulmonary hypertension was found in 3 patients before starting therapy. In the remaining 21 pulmonary artery pressure was within normal limits. In 18 of these an abnormal increase of pulmonary arterial pressure during exercise was found. After 12 months of steroid therapy in all except 2 patients radiological regression was observed. In most patients pulmonary function improved. Normal pulmonary arterial pressure was found in 22 patients. An abnormal increase of pulmonary arterial pressure during exercise was seen in 12 patients. No correlation could be demonstrated between radiological evaluation, respiratory function and effect of steroids on pulmonary circulation.

Sarcoidosis of the lung. Natural history and effects of treatment.

An analysis on the natural course of pulmonary sarcoidosis and effects of corticotherapy was carried out in 960 patients observed during 25 years (1959-1985). There were 434 males and 526 females in the studied group aged from 14 to 69 years, when entering the study. The time of follow-up ranged from 2 to 20 years. In 20% of subjects disease had an acute course, in the remaining 80% the process was symptomless, often of chronic nature. Majority of patients presented with stage I or stage II of the disease (respectively 48.2% and 48.6%). Histological confirmation was obtained in 70% of subjects. Spontaneous remission or improvement was observed in 62.2%. Remission or improvement following treatment was seen in another 18.7% (a beneficial effect being observed in 84.1% of treated patients). Altogether remission or improvement, spontaneous or after treatment, was seen in 80.9% of studied group. In the remaining 19.1%, 2.8% of whom were treated and 16.3% not treated, a progression of the pulmonary changes was observed-the disease evolved into chronic stage. In acute pulmonary sarcoidosis the evolution of the disease was more favorable. Remission or improvement, spontaneous or after treatment, was observed in 89.3% of patients, versus 76.3% of patients with symptomless course of the disease. Recurrence of the disease was observed in 21 cases. It was seen only in treated patients shortly after completion of treatment.