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1.
Sarcoidosis Vasc Diffuse Lung Dis ; 41(1): e2024003, 2024 Mar 26.
Article in English | MEDLINE | ID: mdl-38567556

ABSTRACT

BACKGROUND AND AIM: Sarcoidosis is a granulomatous disorder of unknown etiology characterized by the existence of non-caseating granulomatous inflammation. Diagnosis can be challenging due to the presence of comprehensive clinical, laboratory, and radiologic manifestations. We have evaluated the diagnostic yield of the Kveim test and compared this test with the other conventional laboratory modalities. Our aim was to reach the highest level of diagnostic confidence acknowledging the absolute uncertainty in diagnosis with the current diagnostic enterprises. METHODS: Medical records of 300 sarcoidosis patients were reviewed. Patients were classified into two categories as the conventional laboratory and the Kveim test group to compare the diagnostic yield. RESULTS: Sensitivity of the Kveim test was 76.4% while the conventional laboratory tests provided a 64% diagnostic yield.  The conventional tests had a low diagnostic rate in the early disease stages. Kveim test revealed a high yield diagnosis for all stages of sarcoidosis. Integrated assessment of the two modalities reached a 96.8% sensitivity and a 94,6% specificity. CONCLUSIONS: Conventional laboratory modalities were useful for the assessment of disease activity and identification of organ involvement. Kveim test revealed a significant diagnostic yield for all stages of sarcoidosis. The lowest output was achieved in stage IV patients due to the waning of active granulomatous inflammation.  The highest diagnostic sensitivity was obtained by an integrated analysis of the conventional laboratory and the Kveim test results for all aspects of sarcoidosis.

2.
Monaldi Arch Chest Dis ; 93(3)2022 Oct 21.
Article in English | MEDLINE | ID: mdl-36269206

ABSTRACT

Pleuroparenchymal fibroelastosis (PPFE) is a rare lung disease with unprecedented features characterized by fibroelastotic changes in the subpleural lung parenchyma affecting the upper lobes. PPFE is usually idiopathic, but it can be caused by infection, autoimmunity, bone marrow or lung transplantation, or a genetic predisposition. Histopathologic examination of lung biopsy samples reveals homogenous subpleural fibrosis and abundant elastic fibers, allowing for a definitive diagnosis. As PPFE mimics many interstitial lung diseases, clinicians face significant difficulties in making a definitive final diagnosis. Since most disease-related comorbid conditions manifest at an advanced stage, invasive tissue sampling for histopathologic evaluation is consistently impossible. Such a patient presentation highlights the importance of an analysis based solely on clinical findings, which would provide a definitive diagnosis without the need for a biopsy. Because of its exceptional and inconceivable presentation, PPFE creates a diagnostic dilemma. In light of our two cases and the literature data, we present a diagnostic assessment score assay that relies solely on clinical manifestations without histopathological tissue verification to shed light on the diagnosis of PPFE. This review focuses on PPFE identification through the use of a diagnostic assessment analysis to improve early disease recognition without the use of invasive diagnostic interventions to obtain biopsy samples for histopathologic evaluation. This analytic approach, while not diagnostic in and of itself, may provide a useful pathway for differential diagnosis and may preclude redundant initiatives.


Subject(s)
Lung Diseases, Interstitial , Lung Transplantation , Humans , Tomography, X-Ray Computed , Lung/diagnostic imaging , Lung/pathology , Lung Diseases, Interstitial/diagnosis , Lung Diseases, Interstitial/pathology , Fibrosis
3.
Monaldi Arch Chest Dis ; 91(3)2021 Apr 06.
Article in English | MEDLINE | ID: mdl-33840184

ABSTRACT

Idiopathic pleuropulmonary fibroelastosis is an extremely rare lung disease characterized by the combination of fibrosis of the visceral pleura and the fibroelastotic changes transcending in the subpleural lung parenchyma that predominantly affects the upper lobes with accompanying volume loss. It is mostly idiopathic while infection, autoimmunity, bone marrow or lung transplantation and genetic predisposition may be associated with the development of PPFE. The disease is exceptionally rare as approximately ninety cases have been reported in the literature currently. A 35-year-old female presented with exertional dyspnea, dry cough and weight loss. Physical examination demonstrated platythorax, suprasternal notch deepening and fine rales over the upper lobes. Blood count, serum biochemistry, autoimmunity and serologic markers for collagen vascular diseases were within normal limits. Arterial blood gases demonstrated a low pO2 (48 mm Hg) and a high pCO2 (54 mm Hg) values. Chest x-ray showed bilateral parenchymal fibrotic lesions, left pneumothorax, bronchiectasis in the middle and pleural thickening in the upper lung zones while HRCT revealed bilateral apical pleural thickening, traction bronchiectasis, subpleural reticulations, ground-glass opacities and honeycombing in the upper lobes. Bronchoscopy, BAL cytology, smear and culture did not reveal any pathologic findings. Relevant with the clinical, laboratory, radiologic manifestations and the differential diagnosis with other interstitial lung diseases, PPFE was the final diagnosis. The aim of this case report was to present the clinical manifestations of our case. The second crucial objective was to establish a diagnostic scoring system relevant with the literature and the clinical manifestations of the patient.


Subject(s)
Lung Diseases, Interstitial , Lung Transplantation , Pleural Diseases , Adult , Female , Humans , Lung , Pleura/diagnostic imaging , Pleural Diseases/diagnostic imaging
4.
Monaldi Arch Chest Dis ; 90(4)2020 Dec 23.
Article in English | MEDLINE | ID: mdl-33372740

ABSTRACT

Sarcoidosis is a chronic granulomatous disease of unknown etiology. The disease  most commonly involves the lungs and the mediastinal lymph nodes while extrapulmonary organs such as the skin, eye, liver or spleen may also be comprised. Many imaging modalities have been used for the clinical evaluation of sarcoidosis patients but all have been found to have certain drawbacks for a reliable identification assessment due to the equivocal diagnostic results.  This case series was designed to determine the clinical trenchancy of simultaneous 68Ga citrate PET/CT [Positron emission tomography with 68Ga citrate (68Ga citrate PET/CT)] and 18F-FDG PET/CT [Positron emission tomography with 2-deoxy-2-[fluorine-18] fluoro-D-glucose integrated with computed tomography (18F-FDG PET/CT)] imaging in sarcoidosis patients. The main goal of the study was to evaluate sarcoidosis with respect to disease activity and organ involvement. A total of eight sarcoidosis patients with a comorbid disease suspicion were included in the study. Conventional clinical parameters used for  the diagnosis and the activity of sarcoidosis including CT [Computed tomography (CT)] were compared with the 68Ga-citrate PET/CT findings. Concurrent 18F-FDG PET/CT was performed to verify the granulomatous inflammation of sarcoidosis and to determine coexisting malignant or other inflammatory diseases. Our study results revealed that 68Ga citrate PET/CT imaging appears to be highly useful for the diagnosis, activity assessment and extrapulmonary organ involvement in sarcoidosis. Another crucial finding was the detection of extrapulmonary organ disease that are exceptionally involved, almost inaccessible by biopsy and that could not be otherwise displayed by other conventional imaging modalities. The third hallmark was the identification of a clinically asymptomatic and occult malignancy accompanying sarcoidosis that would not be revealed in any way if synchronous 18FDG PET/CT had not been performed. Simultaneous application of 68Ga citrate and 18FDG PET/CT may provide extremely useful data for the clinical evaluation of sarcoidosis patients in terms of the primary disease diagnosis, activity state, extrapulmonary organ involvement unachievable for biopsy and the clinically occult malignant disorders.


Subject(s)
Fluorodeoxyglucose F18 , Sarcoidosis , Citrates , Humans , Positron Emission Tomography Computed Tomography , Positron-Emission Tomography , Sarcoidosis/diagnostic imaging , Tomography, X-Ray Computed
5.
Monaldi Arch Chest Dis ; 90(3)2020 Jul 29.
Article in English | MEDLINE | ID: mdl-32724228

ABSTRACT

A 67-year-old male presented with cutaneous rash, lassitude and fatigue of three weeks. Personal history included psoriasis and sarcoidosis. Physical examination revealed macular rash on the anterior chest wall. Laboratory results were within normal limits. Chest X-ray showed normal findings. Pulmonary function tests demonstrated a mild obstructive pattern and a mild decrease in DLCO/VA. Thorax CT revealed two nodules in the right upper and middle lobe. 68Ga-citrate PET/CT did not demonstrate any active inflammatory reaction associated with sarcoidosis while 18F-FDG PET/CT revealed increased FDG uptake in the right middle lobe, upper division bronchus and in the left lower abdominal quadrant. Histopathologic examination of the colon biopsy was compatible with adenocarcinoma and bronchoscopic biopsy of the lung lesions revealed nonspecific granulomatous inflammation. BAL cytology was normal while BAL culture did not grow any pathologic organisms. Simultaneous use of 18F-FDG and 68Ga-citrate PET/CT was the hallmark for the final diagnosis in our patient. While FDG/PET has detected the pulmonary and colonic malignant foci in our patient, 68Ga-citrate PET/CT excluded the presence of active granulomatous inflammation of sarcoidosis. Simultaneous utility of these two imaging modalities in patients with sarcoidosis is of great importance in terms of guiding the clinician towards the accurate diagnostic pathway which is the hallmark for final diagnosis, especially in the presence of concomitant malignant disease.


Subject(s)
Adenocarcinoma/secondary , Lung/diagnostic imaging , Neoplasms/diagnosis , Positron Emission Tomography Computed Tomography/methods , Sarcoidosis/diagnosis , Adenocarcinoma/diagnosis , Adenocarcinoma/surgery , Aged , Anthracosis/diagnosis , Anthracosis/pathology , Biopsy , Bronchoscopy/methods , Citrates/metabolism , Colonic Neoplasms/pathology , Diagnosis, Differential , Fluorodeoxyglucose F18/metabolism , Gallium/metabolism , Humans , Lung/metabolism , Lung/pathology , Male , Neoplasms/metabolism , Neoplasms/pathology , Sarcoidosis/complications , Sarcoidosis/metabolism , Sarcoidosis/pathology
6.
Monaldi Arch Chest Dis ; 90(1)2020 Jan 27.
Article in English | MEDLINE | ID: mdl-31986874

ABSTRACT

A 64-year-old male with a history of stabile chronic obstructive pulmonary disease (COPD) presented with increasing dyspnea and sputum for the last two months. Complete blood count showed WBC 14x103/ml, Hgb: 14.2 g/dL and eosinophilia. Blood biochemistry was normal. Chest x-ray showed hyperlucency while thorax computed tomography (CT) revealed obstructive lung disease and bronchiectasis. Pulmonary function tests demonstrated severe obstructive lung disease and a negative bronchoreversibility with a moderately reduced diffusing capacity/alveolar volume (DLCO/VA). ABG gases revealed significant hypoxemia. Sputum culture was negative. Total IgE was 1140 ng/ml. Aspergillus RAST, precipitins and aspergillusgalactomannan antigen were positive. CF genetic screening tests gave negative results. Allergic bronchopulmonary aspergillosis  (ABPA) is a hypersensitivity reaction that occurs due to bronchial aspergillus colonization. It is most common in patients with asthma and cystic fibrosis. We present a COPD case with an acute exacerbation due to Aspergillus fumigatus that lead to an aberrant clinical profile unresponsive to conventional treatment. Clinicians should consider Aspergillus fumigatus as an etiologic agent in an atypical and severe COPD exacerbation.


Subject(s)
Aspergillosis, Allergic Bronchopulmonary/complications , Aspergillosis/complications , Aspergillus fumigatus/immunology , Pulmonary Disease, Chronic Obstructive/etiology , Aspergillosis, Allergic Bronchopulmonary/diagnosis , Aspergillosis, Allergic Bronchopulmonary/drug therapy , Aspergillosis, Allergic Bronchopulmonary/immunology , Bronchiectasis/diagnostic imaging , Disease Progression , Dyspnea/diagnosis , Eosinophilia , Humans , Immunoglobulin E/blood , Male , Middle Aged , Pulmonary Disease, Chronic Obstructive/diagnostic imaging , Pulmonary Disease, Chronic Obstructive/drug therapy , Pulmonary Disease, Chronic Obstructive/physiopathology , Sputum/immunology , Tomography, X-Ray Computed/methods , Treatment Outcome
7.
Monaldi Arch Chest Dis ; 89(3)2019 Oct 18.
Article in English | MEDLINE | ID: mdl-31631644

ABSTRACT

Sarcoidosis is a multisystem granulomatous inflammatory disorder frequently affecting the lungs, but also the liver, along with cirrhosis and portal hypertension occurring in less than 1% of the patients. A 56-year-old female presented with dyspnea, abdominal and leg swelling. Physical examination revealed finger clubbing, ascites and pretibial edema. Chest CT revealed diffuse micronodular opacities in both lungs without any enlarged thoracic lymph nodes. PFTs and DLCO/VA were moderately decreased. Transbronchial biopsy revealed non-caseified granulomas compatible with sarcoidosis. Serologic markers for infectious and autoimmune hepatitis were negative. Liver biopsy showed non-caseating granulomas, severe hepatitis and fibrosis. Stool, urinary analysis and antibodies for Schistosoma infection were negative. Final diagnosis was cirrhosis associated with stage III sarcoidosis. We report a case of sarcoidosis complicated by cirrhosis and portal hypertension with finger clubbing. Clinicians should bear in mind that cirrhosis, portal hypertension and clubbing may arise as the initial manifestations of sarcoidosis.


Subject(s)
Liver Cirrhosis/etiology , Lung Diseases/diagnostic imaging , Osteoarthropathy, Secondary Hypertrophic/diagnosis , Sarcoidosis/complications , Administration, Oral , Anti-Inflammatory Agents/administration & dosage , Anti-Inflammatory Agents/therapeutic use , Ascites/diagnosis , Ascites/etiology , Diuretics/administration & dosage , Diuretics/therapeutic use , Female , Furosemide/administration & dosage , Furosemide/therapeutic use , Humans , Hypertension, Portal/diagnosis , Hypertension, Portal/etiology , Liver Cirrhosis/pathology , Lung Diseases/pathology , Methylprednisolone/administration & dosage , Methylprednisolone/therapeutic use , Middle Aged , Sarcoidosis/diagnostic imaging , Sarcoidosis/drug therapy , Sarcoidosis/pathology , Tomography, X-Ray Computed/methods
8.
Monaldi Arch Chest Dis ; 89(3)2019 Sep 10.
Article in English | MEDLINE | ID: mdl-31505924

ABSTRACT

Sarcoidosis is a multisystemic disease that may lead to neurologic complications in 10% of the patients. Carpal tunnel syndrome is very rare in sarcoidosis. We present two identical twin sarcoidosis patients with carpal tunnel syndrome. A number of factors may cause carpal tunnel syndrome like wrist anatomy, occupation, diabetes, rheumatoid arthritis, pregnancy and renal failure. Although the above factors do not directly cause carpal tunnel syndrome, they may increase your chances of developing or aggravate median nerve damage as it is in sarcoidosis. Sarcoidosis relevant neuropathy and granulomas may be the primary mechanism of sarcoidosis associated carpal tunnel syndrome. Although rare, carpal tunnel syndrome may be a feature of sarcoidosis that may lead to irreversible damage in cases of delayed diagnosis. The presence of this syndrome in identical twin patients may shed light into the pathogenesis and the genetic transmission of sarcoidosis with the associated carpal tunnel syndrome.


Subject(s)
Carpal Tunnel Syndrome/complications , Carpal Tunnel Syndrome/genetics , HLA-DRB1 Chains/genetics , Sarcoidosis/complications , Sarcoidosis/genetics , Adult , Alleles , Female , Humans , Polymorphism, Genetic , Twins, Monozygotic/genetics
9.
Monaldi Arch Chest Dis ; 89(2)2019 Jul 08.
Article in English | MEDLINE | ID: mdl-31282141

ABSTRACT

A 40-year-old female presented with cough, exertional dyspnea, abdominal pain with distention, fatigue, dry eyes and dry mouth. Past history revealed asthma. Physical examination was normal except for tachypnea. We found leukocytosis, azygos fissure on chest X-ray along with normal pulmonary function tests and arterial blood gases.  Thorax computed tomography (CT) revealed bronchiectasis and ground glass opacities in both lungs. Abdominal CT demonstrated thrombosed proximal splenic artery aneurysm. Further diagnostic procedures were done and according to the positive Schirmer test and compatible histopathologic findings of the salivary gland, diagnosis of primary Sjögren's syndrome was established. Splenic artery aneurysm is rare occurring in less than 1% of the population that usually appears as an incidental finding. This is the first case in literature that introduces Sjögren's syndrome as a risk factor for splenic artery aneurysm. The silent presentation of the splenic artery aneurysm should previse the clinicians that such an occurrence may cause a significant diagnostic dilemma.


Subject(s)
Aneurysm/etiology , Sjogren's Syndrome/complications , Splenic Artery/diagnostic imaging , Adult , Aneurysm/diagnostic imaging , Female , Humans , Sjogren's Syndrome/diagnosis , Sjogren's Syndrome/physiopathology , Tomography, X-Ray Computed
10.
Monaldi Arch Chest Dis ; 89(2)2019 Jun 11.
Article in English | MEDLINE | ID: mdl-31181879

ABSTRACT

A 63-year-old male presented with loss of appetite, subfebrile fever, swelling of the right hand and dyspnea on exertion for three months. Past medical history revealed methotrexate treatment of six months for rheumatoid arthritis. Chest radiography and computed tomography (CT) revealed diffuse miliary nodules. PET/CT scan demonstrated diffuse FDG uptake in both lungs, in the spleen, in the right hand, the mediastinal and the axillary lymph nodes. MR of the right hand showed inflammatory arthritis. Histopathology of the right hand tru-cut biopsy revealed degenerative changes. Culture of the hand biopsy tissue was positive for mycobacterium tuberculosis. PET/CT may determine the biopsy and the sampling sites for the early diagnosis of patients with suspected miliary tuberculosis where lesion identification on other modalities may be difficult or unfeasible. High sensitivity for inflammatory diseases makes PET/CT a useful diagnostic utility for enabling early diagnosis in miliary tuberculosis which is a diagnostic predicament.


Subject(s)
Mycobacterium tuberculosis/isolation & purification , Positron Emission Tomography Computed Tomography , Tuberculosis, Miliary/diagnostic imaging , Dyspnea/etiology , Humans , Male , Middle Aged , Sensitivity and Specificity
11.
Monaldi Arch Chest Dis ; 88(3): 981, 2018 11 06.
Article in English | MEDLINE | ID: mdl-30398042

ABSTRACT

A 64-year-old female was admitted for dry cough, dyspnea, fever, loss of appetite, and weight loss. Past medical history revealed scoliosis, cholecystectomy, and Hodgkin lymphoma. ABG values were: pH: 7.42, pCO2: 40.2 mm Hg, pO2: 61.4 mm Hg. Chest CT showed cystic lesions, emphysema, ground glass, and reticular opacities. ABG values worsened under 8L/min nasal oxygen. The patient underwent bilevel positive airway pressure (BiPAP) and methylprednisolone 60 mg/day bid was commenced. The final diagnosis was respiratory insufficiency due to bleomycin toxicity. The patient deceased on the sixth day after transfer to the intensive care unit. Bleomycin is an effective chemotherapeutic agent used for Hodgkin lymphoma treatment. It causes significant lung toxicity in half of the patients. Clinicians should always remember that bleomycin toxicity may lead to fatal complications in patients with comorbid conditions. We present this case to remark the possible consequences of bleomycin toxicity and the precautions taken to preclude bleomycin-induced pulmonary complications are discussed.


Subject(s)
Antibiotics, Antineoplastic/adverse effects , Bleomycin/adverse effects , Hodgkin Disease/drug therapy , Respiratory Insufficiency/chemically induced , Cough/chemically induced , Dyspnea/chemically induced , Fatal Outcome , Female , Humans , Lung/diagnostic imaging , Middle Aged , Radiography, Thoracic , Respiratory Insufficiency/diagnostic imaging , Tomography, X-Ray Computed
12.
Monaldi Arch Chest Dis ; 88(3): 944, 2018 09 11.
Article in English | MEDLINE | ID: mdl-30203633

ABSTRACT

The sensitivity and specificity of exfoliative cell cytology for the diagnosis of exudative pleural effusions varies widely according to the etiologic causes. The aim of this study is to assess the diagnostic value of exfoliative cell cytology for the identification of exudative pleural effusions. This is a retrospective study of the patients with an exudative pleural effusion admitted at our clinic in the last twenty years. We have conducted the clinical, the cytological findings, and the diagnostic results of six hundred patients from hospital records.  Male to female ratio was 2.2:1 with a mean age of 42.8 years (range 18-78 years) among the patients. Samples were processed and evaluated according to the standard methods. Cytology results were reviewed and the patients were stratified according to the final diagnosis of their disease. Of the six hundred exudative effusions, 240 were malignant on exfoliative cytology pleural fluid alone. Adenocarcinoma was the most common type of malignancy. Tuberculosis was the second most frequent etiology for the exudative effusions followed by infection and collagen vascular diseases. Diagnostic accuracy of cytology showed a good correlation with the final diagnosis with an overall 70.1% sensitivity, 62.5% specificity, and a 95.9% positive predictive value for all exudative pleural effusions. Cytologic examination of the pleural fluid is a simple non-invasive procedure as the initial step for the diagnostic work up of patients with a pleural effusion.  Exfoliative cytology provides high a final diagnostic yield for the identification of an exudative pleural effusion etiology. Furthermore, cytologic analysis leads the clinician into the correct diagnostic pathway as the most informative laboratory tool even when it was not diagnostic by itself for equivocal cases.


Subject(s)
Adenocarcinoma/diagnosis , Exudates and Transudates/cytology , Pleural Effusion, Malignant/diagnosis , Pleural Neoplasms/diagnosis , Tuberculosis, Pleural/diagnosis , Adenocarcinoma/pathology , Adolescent , Adult , Aged , Biopsy , Cytological Techniques , Eosinophils , Female , Humans , Leukocyte Count , Lymphocyte Count , Male , Middle Aged , Neutrophils , Pleural Effusion/diagnosis , Pleural Effusion/pathology , Pleural Effusion, Malignant/pathology , Pleural Neoplasms/pathology , Pleural Neoplasms/secondary , Sensitivity and Specificity , Tuberculosis, Pleural/pathology , Young Adult
13.
Monaldi Arch Chest Dis ; 88(1): 910, 2018 Apr 30.
Article in English | MEDLINE | ID: mdl-29741079

ABSTRACT

The main objective of this study was to evaluate the influence of muscle involvement on the clinical features, prognostic outcome, extrapulmonary organ, and endobronchial involvement in sarcoidosis patients. The second aim was to assess the diagnostic yield of muscle biopsy for the histopathologic identification of sarcoidosis.  Fifty sarcoidosis patients participated in the study. The patients were classified into two groups according to the histopathologic presence of non-caseating granulomatous inflammatory pattern of the muscle biopsy samples and were evaluated retrospectively in regard to clinical features, prognosis, extrapulmonary, and endobronchial disease involvement. Pathologic examination of the muscle biopsy samples revealed non-caseating granulomas in eighteen and myositis in seven patients compatible with sarcoidosis. The diagnostic yield of muscle biopsy for demonstrating non-caseating granulomatous inflammation was fifty percent. Patients with muscle sarcoidosis showed a worse prognosis and a more severe extrapulmonary organ involvement than the patients without muscle disease. Muscle biopsy was not statistically significant to delineate diffuse endobronchial involvement while it was suggestive for endobronchial disease clinically. The results of our study reveal that muscle biopsy appears to be a useful diagnostic tool along with its safety and easy clinical applicability. It is a rewarding utility to predict the prognostic outcome and extrapulmonary involvement in sarcoidosis patients. Positive biopsy on the other hand confirms the identification of sarcoidosis in patients with single organ involvement carrying an equivocal diagnostic clinical pattern. Muscle biopsy may be considered as the initial step for the final diagnosis of sarcoidosis in such cases.

14.
Monaldi Arch Chest Dis ; 87(3): 835, 2017 09 27.
Article in English | MEDLINE | ID: mdl-29424190

ABSTRACT

Patients with sarcoidosis usually have a benign course and a favourable prognosis. Although spontaneous remission is common, a progressive disease with a severe prognosis occurs in a small but significant number of patients. The aim of this study was to evaluate the potential significance of HLA antigens as a clinical marker on the outcome of sarcoidosis patients. We conducted a retrospective cohort study for HLA class I and II allels in 74 sarcoidosis patients and 72 healthy transplant donors. Bronchoscopy and bronchial biopsies were performed in each patient. Two or more positive bronchial biopsy samples revealing granulomatous inflammation was defined as diffuse while one positive biopsy sample was considered as limited endobronchial disease. Three or more extrapulmonary organ involvement was denoted as severe extrapulmonary disease. The patients were followed-up at least for eight years.  Incidence of progressive disease was significantly high in patients with positive HLA-DRB1*07, DRB1*14 (p<0.05) and DRB1*15 (p <0.001) allels. HLA-DRB1*14 and DRB1*15 were associated with severe extrapulmonary organ involvement (p<0.001). HLA-DRB1 *14 (p<0.05) and DRB1*15 (p<0.001) were significantly more frequent in patients with diffuse endobronchial involvement. Incidence of familial disease was 14.8% with a 6.7% identical HLA typing. Presence of HLA class I and II allels may influence the severity and prognosis of sarcoidosis significantly. Apart from defining genetic susceptibility, HLA class I and class II allels appear to be relevant and crucial markers for the to predict the clinical outcome of sarcoidosis. Distinct heterogenity of sarcoidosis may arise from the particular presence of different allels in invidual patients.


Subject(s)
Genetic Predisposition to Disease/genetics , HLA Antigens/genetics , HLA-DRB1 Chains/genetics , Sarcoidosis, Pulmonary/genetics , Adult , Alleles , Bronchoscopy/methods , Disease Progression , Female , HLA Antigens/immunology , HLA-DRB1 Chains/immunology , Humans , Incidence , Male , Outcome Assessment, Health Care , Prognosis , Retrospective Studies , Sarcoidosis, Pulmonary/diagnostic imaging , Sarcoidosis, Pulmonary/mortality , Sarcoidosis, Pulmonary/physiopathology , White People/ethnology
15.
Monaldi Arch Chest Dis ; 87(3): 893, 2017 12 21.
Article in English | MEDLINE | ID: mdl-29424195

ABSTRACT

Sarcoidosis is a systemic disease characterized by noncasefied granulomas in various organs. Incidence of splenic disease is variable and is reported to occur in 6.7 to 77 percent of the patients. Firm data establishing the clinical features and the association of splenic involvement with prognosis in sarcoidosis is scant. The aim of our study was to investigate the clinical features and the consequence of splenic involvement on the prognostic outcome of sarcoidosis patients. We evaluated the clinical and laboratory findings in 82 sarcoidosis patients. Forty-two patients with splenic involvement were compared to 48 sarcoidosis patients without splenic disease in regard to laboratory findings, endobronchial disease, extrapulmonary organ involvement, and prognosis. Lung biopsy sample was considered positive if it demonstrated noncaseating granulomas with negative fungal and mycobacterial cultures. Splenic sarcoidosis was identified by ultrasound or computed tomography and was designated as limited, diffuse or without splenic involvement. Extrapulmonary organ sarcoidosis was classified as extensive and limited. Endobronchial disease was categorized as limited or diffuse involvement. The most commonly comprised organ was lung in 95% of the cases followed by lymph nodes, skin, eye, spleen and liver in the order of frequency. Splenic disease was diffuse in 22 patients. Of these patients, 14 had extensive extrapulmonary organ involvement while 16 had diffuse endobronchial disease. There was no significant difference between the three groups for FEV1, FVC, TLC, DLCO/VA, serum and 24h urinary calcium levels. Serum ACE was higher in patients with diffuse splenic involvement (p<0.001). Incidence of persistent chronic disease was significantly higher (p<0.001) in patients with diffuse splenic sarcoidosis. Extensive extrapulmonary organ involvement and diffuse endobronchial disease were more common (p<0.001) in this group. Extensive extrapulmonary organ involvement and diffuse endobronchial disease were more frequent in patients with diffuse splenic sarcoidosis. Patients with diffuse splenic granulomas had a worse prognosis than the patients without splenic involvement or patients with limited splenic disease. Diffuse splenic involvement emerges to be a significant risk factor for persistent chronic sarcoidosis. Extensive granuloma burden in an organ may be the decisive clinical marker for the prognostic outcome of sarcoidosis patients.


Subject(s)
Granuloma/pathology , Lung/pathology , Sarcoidosis/complications , Spleen/pathology , Splenic Diseases/pathology , Adult , Biomarkers , Bronchial Diseases/pathology , Bronchoscopy/methods , Chronic Disease , Female , Humans , Incidence , Lung/physiopathology , Male , Prognosis , Respiratory Function Tests/methods , Retrospective Studies , Risk Factors , Sarcoidosis/mortality , Sarcoidosis/pathology , Sarcoidosis/physiopathology , Splenic Diseases/diagnostic imaging , Splenic Diseases/epidemiology , Tomography, X-Ray Computed/methods , Ultrasonography/methods
16.
Multidiscip Respir Med ; 8(1): 26, 2013 Mar 22.
Article in English | MEDLINE | ID: mdl-23521826

ABSTRACT

BACKGROUND: Sarcoidosis is a systemic disease characterized by the formation of noncaseating granulomas in various tissues. Cutaneous involvement occurs in 20 to 35 percent of the patients and may be the initial manifestation of the disease. Our study was performed to discriminate the clinical, laboratory, and prognostic differences between patients with specific and nonspecific cutaneous involvement. The second aim was to asses the diagnostic usefulness of punch biopsy in sarcoidosis. METHODS: The clinical, laboratory, pathological features, and skin biopsy results of 120 patients with cutaneous sarcoidosis were evaluated. The patients fulfilled clinical, radiologic or both features of sarcoidosis supported by the histopathologic evidence of noncaseating granulomas.Skin involvement was the initial finding in 30% of the patients. Erythema nodosum and lupus pernio were the most common skin lesions. Almost all of the patients with LP were either stage 0 or 1. Respiratory symptoms occurred in 72.2% of the patients with specific skin involvement. BronchoalveolarLavage (BAL) lymphocytosis, high ratio of CD4/CD8 and elevated serum Angiotensin Converting Enzyme (ACE) were more frequent in patients with specific cutaneous lesions. The frequency of progressive disease was significantly higher in this group. Punch skin biopsy was diagnostic in 81.6% of the patients with a complication rate of 4%. CONCLUSIONS: Specific cutaneous lesions along with BAL lymphocytosis, high CD4/CD8 ratio and elevated serum ACE levels may be predictors of progressive disease in sarcoidosis. Punch biopsy is a simple technique with a high diagnostic yield and a low complication rate for cutaneous sarcoidosis.

17.
Multidiscip Respir Med ; 7(1): 14, 2012 Jul 17.
Article in English | MEDLINE | ID: mdl-22958435

ABSTRACT

A 27 year old female with Graves' disease presented with fever, exertional dyspnea and polyarthralgia. Erythema nodosum had occured three months earlier. The patient declared irregular use of propylthiouracil (PTU) for the last 8 months. Neutropenia and microscopic hematuria developed in the second week of admission. Chest X-ray showed inhomogenous pulmonary opacities, left pleural effusion and cardiomegaly. Computed tomography (CT) revealed multiple subpleural nodules, left pleural effusion, pericardial effusion, enlarged mediastinal and axillary lymph nodes. Bronchoalveolar lavage (BAL) cytology demonstrated hemosiderin laden macrophages. Histopathologic examination of the transbronchial biopsy specimen revealed a nonspecific inflammation. Serum was positive for ANA, P-ANCA, MPO-ANCA, PR3-ANCA and negative for anti-ds-DNA, C-ANCA, C3, C4 and anti-histone antibody. All symptoms resolved in two months after PTU withdrawal and starting steroid treatment. The same clinical manifestations recurred when the patient used PTU erronously one month after discharge.This is a case of PTU induced-autoimmune disease in whom the accurate distinction between drug-induced-lupus (DIL) and vasculitis was not possible due to the significant overlap of clinical and laboratory findings causing a significant diagnostic challenge for the chest physician.

18.
Multidiscip Respir Med ; 6(3): 180-2, 2011 Jun 30.
Article in English | MEDLINE | ID: mdl-22958304

ABSTRACT

Round atelectasis is a benign inflammatory condition most frequently observed in patients with asbestos exposure but it can also result from a variety of chronic pleural diseases like infection. It has not previously been described in sarcoidosis. We report the occurrence of round atelectasis in four previously diagnosed sarcoidosis patients who were under follow up at our outpatient clinic. Three patients had symptoms consisting of thoracic pain, dry cough and sensation of fullness at the posterior thorax, respectively. Chest roentgenogram showed subpleural or pleural based opacity with diameters ranging from 2 to 3 cm in each of the patients. Chest computerized tomography (CT) revealed features of round atelectasis. Fiberoptic bronchoscopy with transbronchial lung biopsy was performed. Diagnosis was confirmed by the histopathologic examination of the biopsy samples. The mechanical influence of a prior pleural effusion due to sarcoidosis may be the predominant mechanism underlying the onset of round atelectasis in these patients. Clinicians should bear in mind the possibility of sarcoidosis as an etiologic factor for round atelectasis.

19.
Multidiscip Respir Med ; 6(4): 226-31, 2011 Aug 31.
Article in English | MEDLINE | ID: mdl-22958450

ABSTRACT

BACKGROUND AND AIMS: Anxiety and depression are common in patients with chronic obstructive pulmonary disease (COPD). The degree of lung function may not explain anxiety and depression. The aim of our study was to assess the psychological aspects of COPD, to test the BODE index (a composite score of body mass, obstruction, dyspnea and exercise capacity), and to evaluate the association between atypical cytologic findings of sputum, bronchoalveolar lavage (BAL) and the pyschological components of the disease. METHODS: COPD was classsified according to the GOLD stages based on forced expiratory volume in 1 second (FEV1) in 60 stable patients. The BODE index was calculated for grading COPD. The Hospital anxiety and depression (HAD) scale was used to appraise the anxiety and depression symptoms. Cytologic examination of sputum and BAL samples were performed in each patient. The cytologic findings were classified as normal, mild, moderate or severe atypia. RESULTS: The overall prevalance of anxiety and depression symptoms was 41.7% and 46.7% respectively. The prevalance of these symptoms increased with increasing BODE stages and correlated well with the severity of atypical BAL cytology results (p < 0.001). Dyspnea and reduced exercise capacity were the predominant mechanisms leading to anxiety and depression symptoms associated with COPD. CONCLUSIONS: We conclude that the BODE index is superior to GOLD stratification for explaining anxiety and depression symptoms in COPD. BAL cytologic findings, which reflect the distal parenchymal lung structure, correlated significantly with the presence of the anxiety and depression symptoms.

20.
Multidiscip Respir Med ; 5(4): 264-6, 2010 Aug 31.
Article in English | MEDLINE | ID: mdl-22958601

ABSTRACT

A 51-year-old man with Behçet's disease complained of fever, dry cough and dyspnea during exertion. Chest CT showed ground glass opacities with interstitial septal thickening in both lungs. Bronchoalveolar lavage (BAL) revealed amorphous and lipoproteinaceous material that was periodic acid-Schiff (PAS) stain positive. Transbronchial biopsy specimen demonstrated PAS positive alveolar eosinophilic material consistent with pulmonary alveolar proteinosis. Serum anti-granulocyte-macrophage colony stimulating factor (GM-CSF) antibody was negative. Recent studies have reported anti-GMCSF not present in the the serum of patients with secondary pulmonary alveolar proteinosis (PAP) but they have not reported so in patients with idiopathic PAP. We report a case of alveolar proteinosis in the setting of Behçet's disease with spontaneous remission.

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