Phenotypes of organ involvement in sarcoidosis.

Sarcoidosis is a highly variable, systemic granulomatous disease of hitherto unknown aetiology. The GenPhenReSa (Genotype-Phenotype Relationship in Sarcoidosis) project represents a European multicentre study to investigate the influence of genotype on disease phenotypes in sarcoidosis.The baseline phenotype module of GenPhenReSa comprised 2163 Caucasian patients with sarcoidosis who were phenotyped at 31 study centres according to a standardised protocol.

[Combined pulmonary fibrosis and emphysema syndrome].

CPFE-combined pulmonary fibrosis and emphysema is a new term for a syndrome whose main characteristic is fibrosis in lower pulmonary lobes with simultaneous emphysema in upper pulmonary lobes. CPFE patients have well preserved pulmonary test values for unexpectedly long period, but extremely lowered carbon monoxide diffusion capacity and significant arterial hypertension. All CPFE studies indicate that CPFE occurs predominately in older male population.

WHOQOL-bREF questionnaire as a measure of quality of life in sarcoidosis.

The aim of this study was to evaluate the usefulness of a short form of the WHOQOL questionnaire, the WHOQOL-BREF, which consists of 24 questions, in evaluating quality of life (QOL) in sarcoidosis patients. A group of 97 sarcoidosis patients and a matched group of 97 healthy controls took part in the study. Their QOL was examined by means of the WHOQOL-BREF, and the respiratory functions were measured in sarcoidosis patients.

Lung lavage cell profiles in diffuse lung disease.

The standard armamentarium of tests that are used by pulmonologist are laboratory tests, pulmonary function tests, different radiological techniques (conventional chest-X rays, HRCT scans, etc) and pathohistological analyses of biopsies. The minimally invasive bronchoalveolar lavage (BAL) procedure, in addition to methods earlier mentioned, is an important diagnostic instrument that can facilitate the diagnosis of various diffuse lung diseases (DLD). BAL fluid white blood cell profiles are analyzed, malignant cells looked for, and in certain circumstances particular stains are performed to detect yet other cell types.

Morphometric and DNA image analysis of bronchoalveolar lavage fluid macrophages nuclei in interstitial lung diseases with lymphocytic alveolitis.

Lymphocytic alveolitis is a characteristic of diverse interstitial lung diseases (ILD-s), but macrophages are often more numerous cell population in bronchoalveolar lavage fluid (BALF). Aim of this study is to analyze morphometric characteristics of macrophages nuclei in BALF in patients with ILD-s and to detect possible differences allowing distinguishing sarcoidosis from other lymphocytic alveolitis ILD-s. Thirty-one patient with interstitial lung disease who had lymphocytic alveolitis in BALF cell count (17 sarcoidosis and 14 other ILD-s) and nine controls were included in the study.

[Diffuse lung disease--advances in patient management].

Diffuse lung diseases (DLD), known as interstitial lung diseases or diffuse parenchymal lung diseases, are a large group of disorders of diverse etiology and causes, however, sharing similar clinical, radiological and pathophysiological characteristics. In the last fifteen years, DLD have attracted considerable interest of medical society. During that period, a consensus of the British Thoracic Society on the Diffuse Parenchymal Lung Disease and Statement of the American Thoracic Society (ATS) and European Respiratory Society (ERS) on idiopathic pulmonary fibrosis, idiopathic interstitial pneumonias and sarcoidosis have helped precisely define certain phenotypes.

Diffuse pulmonary ossification: an unusual interstitial lung disease.

Purpose Of Review: Diffuse pulmonary ossification is a rare disease characterized by diffuse small bone fragments in the lung tissue. It can be idiopathic or associated with underlying chronic pulmonary or heart diseases. The majority of cases had been diagnosed on autopsy.

Hypereosinophilic syndrome: diagnosis and treatment.

Purpose Of Review: Hypereosinophilic syndrome is increasingly recognized as a heterogeneous group of disorders, in some cases with precisely defined pathogenesis, which has led to changes in diagnostic approaches and therapeutic strategies. An update on causes and modern therapy is presented here.

Recent Findings: Clonal eosinophilias belong to the group of myeloid malignancies.

Epidemiological characteristics of sarcoidosis patients hospitalized in the Uuniversity Hospital for Lung Diseases "Jordanovac" (Zagreb, Croatia) in the 1997-2002 period.

The aim of our study was to explore the characteristics of hospitalized patients with sarcoidosis concerning age, gender, clinical forms and staging, seasonality, geographical distribution, smoking habit and profession, familial clustering and mortality. We included 476 biopsy-proven sarcoidosis patients who were diagnosed at the University Hospital for Lung Diseases "Jordanovac" in the period from 1997-2002. Most of the patients (44%) were in the group of age between 20 and 40 years.

Fatigue is associated with quality of life in sarcoidosis patients.

Background: Fatigue is one of the core symptoms of sarcoidosis patients. Although it is known that fatigue affects quality of life (QOL) in other patient groups, this relationship has never been studied in sarcoidosis patients using a reliable and valid fatigue scale and a multidimensional QOL instrument. The present cross-sectional study among sarcoidosis patients attempts to gain more insight into this relationship.

Relationship between symptoms and quality of life in a sarcoidosis population.

Background: As sarcoidosis is a multisytemic disorder, patients may suffer from various symptoms. The relationship between frequently reported symptoms and quality of life (QOL) has not yet been studied.

Objectives: The aim of the present cross-sectional study was to examine the predictive value of the most frequently reported subjective symptoms on QOL after controlling for demographic variables and clinical parameters.

Psychometric qualities of the Fatigue Assessment Scale in Croatian sarcoidosis patients.

Background And Aim: Fatigue is an unspecific symptom, but a major problem in sarcoidosis patients. There is a need for a reliable and valid way to measure fatigue. The Fatigue Assessment Scale (FAS) has good psychometric properties in healthy and sarcoidosis samples in the Netherlands, but nothing is known about the psychometric qualities of the FAS in sarcoidosis samples from other countries.

Multiple primary intrathoracic neoplasms: case report and a review of the literature.

This is a case report of a patient diagnosed with three distinct primary intrathoracic tumors (mesothelioma, carcinoid and B-cell lymphoma). The patient had previously had mycosis fungoides. The occurrence of multiple neoplasms in a single patient, synchronous or metasynchronous, is not a rare phenomenon; the incidence varies from 1-11% of all neoplasms.

Prevalence of hospitalized patients with sarcoidosis in Croatia.

The aim of this study was to investigate the prevalence of hospitalized patients of sarcoidosis in the Republic of Croatia, its distribution in relation to sex and age as well as its geographical distribution. The data on sarcoidosis patients hospitalized in Croatia in the last six years, from 1997 to 2002, were analyzed retrospectively. The prevalence of sarcoidosis patients hospitalised in the Republic of Croatia is 4.

The role of transbronchial lung biopsy in the diagnosis of solitary pulmonary nodule.

Transbronchial lung biopsy (TBLB) is a well-recognized diagnostic technique in diffuse interstitial lung diseases, but it is not considered to be the first choice in investigation of solitary pulmonary nodules (SPN). The main idea of this study was to increase the sensitivity of bronchoscopy using multiple techniques, especially TBLB, thus to avoid more aggressive diagnostic procedures. The objective of this prospective study was to evaluate the efficacy and safety of TBLB in the diagnosis of SPN, in comparison with other bronchoscopic techniques.

[Bronchoalveolar lavage].

Bronchoalveolar lavage (BAL) is a bronchoscopic technique that reveals specific insight in the distal parts of lung parenchyma. During the past twenty years, research of cellular and extracellular bronchoalveolar profiles gave important information on pathogenesis of some pulmonary disorders, promoting this technique as a diagnostic tool in pulmonary infections and interstitial lung diseases. Bronchoalveolar lavage is a safe, well-tolerated and suitable diagnostic procedure in immunocompromised patients.

[BOOP-bronchiolitis obliterans organizing pneumonia].

Bronchiolitis obliterans organizing pneumonia (BOOP) is increasingly recognized in "Jordanovac" Clinical hospital for lung diseases. The characteristic pathohistologic finding is the granulated tissue plugs within the lumen of small airways which extend into the alveolar ducts and alveoli. Numerous conditions and diseases show such histologic picture but the clinician by means of clinical, radiologic and laboratory findings establishes the final diagnosis.

[Early recognition of Churg-Strauss syndrome].

We present a patient with Churg-Strauss syndrome which appeared with asthma, chronic sinuitis, peripheral and tissue oeosinophilia, migratory pulmonary infiltrates and polyneuropathy but without histologically proven granuloma or vasculitis. The patient successfully recovered under corticosteroid therapy. The aim of this presentation is early recognition of Churg-Strauss syndrome in order to timely diagnose and treat these patients.