Imaging Plays a Key Role in the Diagnosis and Control of the Treatment of Bone Sarcoidosis.

Sarcoidosis is a multisystem granulomatous disease of unknown origin. The most frequent localizations are thoracic lymph nodes and/or parenchymal lung disease, nevertheless any other organ may be involved. Musculoskeletal sarcoidosis, previously considered a rare manifestation of the disease, is presently recognized with increasing frequency, due to the development of modern imaging modalities. The classical X-ray sign of bone sarcoidosis is the image of lace in the phalanges of the hands. Most other locations present with atypical radiological images. Therefore, they may mimic metastatic neoplastic disease, especially when they are the first sign of sarcoidosis not previously recognized. On such occasions, none of the imaging methods will give the correct diagnosis, histopathological verification, monitoring of lesions or clinical data in a patient with confirmed sarcoidosis are indicated. The article summarizes the current status of knowledge concerning the recognition and therapy of bone sarcoidosis. In addition, an illustrative case of patient with bone and bone marrow sarcoidosis is presented.

New 6-Minute-Walking Test Parameter-Distance/Desaturation Index (DDI) Correctly Diagnoses Short-Term Response to Immunomodulatory Therapy in Hypersensitivity Pneumonitis.

The six-minute-walking test (6MWT) is an easy-to-perform, cheap and valuable tool to assess the physical performance of patients. It has been used as one of the endpoints in many clinical trials investigating treatment efficacy in pulmonary arterial hypertension and idiopathic pulmonary fibrosis. However, the utility of 6MWT in patients diagnosed with hypersensitivity pneumonitis (HP) is still under investigation. The aim of the present retrospective study was to assess the value of different 6MWT parameters, including the newly developed distance-desaturation index (DDI), to evaluate immunomodulatory treatment outcomes in HP patients. METHODS: 6MWT parameters (distance, initial saturation, final saturation, desaturation, distance-saturation product (DSP), and DDI) were analyzed at baseline and after 3 to 6 months of treatment with corticosteroids alone or in combination with azathioprine. RESULTS: 91 consecutive HP patients diagnosed and treated in a single pulmonary unit from 2005 to 2017 entered the study. There were 44 (48%) males and 52 (57%) patients with fibrotic HP (fHP). Sixty-three patients (69%) responded to treatment (responders) and 28 (31%) did not respond (non-responders). In the responders group, all parameters assessed during 6MWT significantly improved, whereas in non-responders, they worsened. Medians (95% CI) of best indices were post-treatment DDI/baseline DDI-1.67 (1.85-3.63) in responders versus 0.88 (0.7-1.73) in non-responders (p = 0.0001) and change in walking distance-51 m (36-72 m) in responders, versus 10.5 m (-61.2-27.9) in non-responders (p = 0.0056). The area under the curve (AUC) of receiver operating characteristics (ROC) for post-treatment DDI/baseline DDI was 0.74 and the optimal cut-off was 1.075, with 71% of specificity and 71% of sensitivity. CONCLUSIONS: 6MWT may be used as a tool to assess and monitor the response to immunomodulatory therapy in HP patients, especially if indices incorporating both distance and desaturation are used. Based on the present study results, we recommend 6MWD and DDI use, in addition to FVC and TL,co, to monitor treatment efficacy in patients with interstitial lung diseases.

Dominating Cause of Pulmonary Hypertension May Change Over Time-Diagnostic and Therapeutic Considerations in a Patient with Pulmonary Hypertension Due to Rheumatoid Arthritis with Lung Involvement.

Chronic lung diseases are one of the most frequent causes of pulmonary hypertension (PH). The diagnostic challenge is to differentiate PH due to chronic lung disease from pulmonary arterial hypertension (PAH) with coexisting chronic lung disease. Moreover, the dominating cause of PH may change over time, requiring the implementation of new diagnostic procedures and new treatment modalities. We present a 68-year-old female, initially diagnosed with PH in the course of interstitial lung disease, with restrictive impairment of lung function. Therapy with immunosuppressive drugs resulted in significant clinical, radiological and functional improvement. However, five years later, arthritis symptoms developed, with PH worsening, despite stable lung disease. The patient was diagnosed with PAH in the course of rheumatoid arthritis. The introduction of sildenafil resulted in marked clinical and hemodynamic responses. Long-term survival (eleven years from PH onset and five years from PAH confirmation) has been achieved, and the patient remains in good functional condition. As the differential diagnosis of PH in patients with lung diseases is complex, the cooperation of pulmonologists and cardiologists is mandatory to obtain therapeutic success.

Correlation of bronchoalveolar lavage lymphocyte count with the extent of lung fibrosis and with plethysmographic lung volumes in patients with newly recognized hypersensitivity pneumonitis.

INTRODUCTION: Hypersensitivity pneumonitis (HP) is an increasingly recognized interstitial lung disease, presenting with elevated total cell counts and high percentage of lymphocytes in bronchoalveolar lavage fluid (BALF). Despite many publications, there is no consensus in the literature concerning BALF cellular composition in patients with prolonged course of HP. AIM: The aim of the present retrospective study was to investigate the influence of disease duration, smoking habits, and the extent of lung fibrosis on BALF cells' population in patients with newly recognized HP. MATERIAL AND METHODS: In total, 94 patients (49 females, 45 males), mean age 52 (±12) years, with HP recognized according to recently proposed criteria, were enrolled into the present study. Chest CT scans were retrospectively reviewed by two independent radiologists. BALF evaluation was performed as a part of routine diagnostics according to recent recommendations. RESULTS: Percentage of lymphocytes in BALF was significantly lower in patients with lung fibrosis (stage 1 and 2) comparing to those without lung fibrosis (stage 0). Significant correlation was also found between the percentage of BALF lymphocytes and plethysmographic lung volumes, but not with lung transfer capacity for carbon monoxide (TLCO% pred). Smoking did not influence BALF results in our study group. CONCLUSIONS: BALF lymphocytosis correlated with the presence and the extent of lung fibrosis on chest CT as well as with plethysmographic lung volumes but did not with TLCO and smoking habits in newly recognized HP pneumonitis.

Hypersensitivity pneumonitis recognised in a single pulmonary unit, between 2005 and 2015 - comparison with recently proposed diagnostic criteria.

INTRODUCTION: Hypersensitivity pneumonitis (HP) is the third most common interstitial lung disease after idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia. Pathogenesis of HP is related to repeated exposure to inhaled environmental antigens that sensitise the susceptible, genetically predisposed persons. The aim of the present retrospective study was to summarise the diagnostic methods used in consecutive patients with HP, recognised in a single pulmonary unit, between 2005 and 2015, and to compare them with current diagnostic criteria. MATERIAL AND METHODS: 135 patients, 68 males, 67 females, median age 53 years (18-75 years), entered the study. Chest CT features characteristic of HP were defined as: mosaic attenuation of lung parenchyma, air trapping and/or ill-defined centrilobular nodules. Lymphocytosis in BAL was defined as ≥ 30%. RESULTS: Median time from first symptoms to diagnosis was 12 months. The exposure to one or more allergens was found in 94% of patients, chest CT features characteristic of HP have been reported in 87%, BAL lymphocytosis - in 86%. According to recent diagnostic criteria - in 54% of patients, clinical diagnosis of HP was confident, in 16% - probable, in 26% - possible and in 4% - unlikely. The confirmation of HP with lung biopsy has been obtained in 36% of non-confident cases (16% of the study group). CONCLUSION: HP diagnosis was confirmed according to current diagnostic criteria in 70% of patients diagnosed between 2005 and 2015. Contradictions to lung biopsy have been the main reason for inability to confirm HP in non-confident cases.

The value of serum precipitins against specific antigens in patients diagnosed with hypersensitivity pneumonitis - retrospective study.

INTRODUCTION: Hypersensitivity pneumonitis (HP) is the third most common interstitial lung disease, and is often under-recognized, especially in patients who are not aware of their occupational or environmental contact with organic antigens. The aim of the present study was to assess the results of serum specific IgG antibodies (ssIgG) in HP patients and their correlation with clinical data. MATERIAL AND METHODS: 128 HP patients, median age 53 years, participated in the study. The control group consisted of 102 patients with interstitial lung diseases (ILDs) other than HP. Assessment of pretreatment ssIgG to thermophilic actinomycetes and protein antigens from bird droppings (pigeons, hens, ducks, parrots, turkeys) was performed by double diffusion in agar gel according to Ouchterlony method. RESULTS: Positive precipitins were obtained in 57% of all HP patients and in 61% of those exposed to above mentioned antigens. Positive results in the control group were obtained in 7% of patients. Sensitivity of ssIgG in HP group was 0.57 and specificity 0.93. Precipitins to at least one bird antigen was confirmed in 64% of HP patients exposed to birds. Precipitins to thermophilic bacteria were found in 29% of HP patients exposed to hay or hay products. CONCLUSIONS: The results of the study indicate that ssIgG against birds' allergens were the valuable diagnostic tool in HP patients. Low-rate of confirmation of ssIgG to thermophilic bacteria in patients exposed to hay or hay products indicate that other microorganisms, most likely molds, could be responsible for the disease development.

Diagnostic Difficulties in Bone Sarcoidosis Imaging. A Cases Study.

Sarcoidosis is a multiorgan inflammatory disease that rarely involves the musculoskeletal system. A typical radiographic presentation is only noted with phalangeal lesions in the hands and feet, and other skeletal sites of sarcoidosis are a diagnostic imaging challenge [1]. We describe two cases of patients with sarcoidosis in whom pathologic bone marrow lesions were diagnosed on MRI scans. The magnetic resonance findings were non-specific and metastatic lesions or multiple myeloma were suspected. The case analysis serves to point to limitations of imaging studies in diagnosing bone sarcoidosis and underline the importance of cooperation between the radiologist and the clinician. The role of magnetic resonance imaging in the diagnostic algorithm for bone sarcoidosis should mostly focus on locating lesions, indicating biopsy sites and follow-up of abnormalities.

[Hypersensitivity pneumonitis: Diagnostic criteria, treatment, prognosis and prevention]. / Alergiczne zapalenie pecherzyków plucnych - kryteria rozpoznania, leczenie, rokowanie i zapobieganie.

Hypersensitivity pneumonitis (HP) is caused by inhalation of environmental antigens. Farmers and bird keepers are most frequently affected by this desease. The HP diagnosis is based on clinical symptoms (cough, dyspnea) in a person exposed to environmental antigens, and the presence of characteristic changes in high resolution chest computed tomography (HRCT) (bilateral, mosaic, ground glass opacities in the middle and lower lung zones, ill-defined centrilobular nodules and the sign of air-trapping on expiration). This type of HRCT pattern is most frequently found in the patients with subacute HP. Bronchioloalveolar lavage fluid (BALF) examination is helpful in establishing the HP diagnosis, when the increased total number of cells, with the predominance of T lymphocytes (> 50%), and the increased number of neutrophils (> 3%) and mastocytes (> 1%) are found. The presence of specific serum precipitins increases the likelihood of HP. In case of atypical clinical presentation, lung biopsy is recommended. The diagnostic criterion of HP is the presence of ill-defined non-necrotising granulomas, after excluding other granulomatous lung diseases. The prevention and treatment of HP is based on the elimination of the antigen from the environment. Corticosteroids may contribute to the improvement in the acute and sub-acute form of the disease but their long term effectiveness is uncertain. The prognosis of HP patients is generally perceived as good, especially in those patients in whom antigen avoidance is possible. Nevertheless, in some patients progressive pulmonary fibrosis and development of severe respiratory insufficiency is observed. Med Pr 2016;67(4):517-527.

Non-high risk PE in the patients with acute or exacerbated respiratory disease: the value of the algorithm based on D-dimer evaluation and Revised Geneva Score.

INTRODUCTION: The diagnostic algorithm of non-high risk pulmonary embolism (PE) is based on probability scoring systems and plasma D-dimer (DD) assessment. The aim of the present study was to investigate the efficacy of Revised Geneva Scoring (RGS) and DD testing for the excluding of non-high risk PE, in the patients admitted to the hospital due to acute respiratory diseases. MATERIAL AND METHODS: The consecutive patients, above 18 years of age, referred to the department of lung diseases, entered the study. The exclusion criteria were: the pregnancy and the suspicion of high risk PE. Plasma DD was measured with quick ELISA test, VIDAS D-dimer New, bioMerieux, France. Multislice computed tomography angiography was performed in all of the patients. RESULTS: 153 patients, median age 65 (19-88) years entered the study. The probability of PE was: low - in 58 patients (38%), intermediate - in 90 (59%), high - in 5 (3%). DD < 500 ng/ml was found in 12% of patients with low and intermediate probability of PE. PE was recognized in 10 out of 153 patients (7%). None of the patients with DD < 500 ng/ml was diagnosed with PE (NPV 100%). Median DD value was significantly higher in PE patients comparing to non-PE (4500 ng/ml and 1356 ng/ml respectively, p = 0.006). CONCLUSION: In the group of the patients with acute respiratory symptoms, low or intermediate clinical probability scoring combined with normal DD had a high NPV in excluding PE. Nevertheless, such approach was not very effective, as the increased DD was noted in 88% of the examined population.

[Interstitial lung disease in patients with primary biliary cirrhosis]. / Zmiany sródmiazszowe w plucach u chorych na pierwotna zólciowa marskosc watroby.

Primary biliary cirrhosis (PBC) is a chronic autoimmune disorder of unknown etiology. The disease affects middle-aged women and is characterized by the destruction of the intralobular bile ducts that causes consequent cholestasis. AMA is a hallmark of PBC, composed mostly of IgG and IgM class. The M2 antibody is the most specific one, with sensitivity range of 54-98% depending on type of test used. PBC is often accompanied by other autoimmune diseases, such as Sjögrens syndrome, thyroiditis, rheumatoid arthritis, dermatomyositis, polymyositis. Interstitial lung disease (ILD) has been reported in patients with primary biliary cirrhosis but its frequency and nature are poorly understood. We report pulmonary involvement in the course of PBC in 4 middle-aged women. Histopatological examination of lung specimens was available in three patients: two presented with sarcoid - like granulomas, one with lymphocytic interstitial pneumonia (LIP). In one patient the diagnosis of pulmonary fibrosis was based on clinical and radiological features. Because of abnormal pulmonary function tests (PFT) results all the patients were treated with prednisone, one, additionally with azathioprine. The treatment was successful in all of the patients.