Clinical outcomes of radical radiotherapy for pulmonary sarcomatoid carcinoma

Purpose@#Pulmonary sarcomatoid carcinoma (PSC) is recognized for its aggressiveness and poor prognosis. The role of radical radiotherapy in PSC remains uncertain due to its scarcity and limited data. In the absence of an effective systemic agent, this study aims to explore the possibility of cure and to investigate potential prognostic factors and treatment outcomes. @*Materials and Methods@#From January 2005 to December 2021, 149 PSC patients were identified. Among 62 patients who received radiotherapy for lung lesions, 25 who underwent palliative radiotherapy and 16 who underwent surgery were excluded. @*Results@#The median patient age was 71 years. The majority were male, and 17 patients (81.0%) were diagnosed at an advanced stage. After radical radiotherapy, distant metastasis (47.6%) was the most common site of failure, while the local recurrence rate was quite low (9.5%). Eventually, five patients (26.3%) demonstrated either a partial response or complete remission, including three complete remissions with durable responses. The median progression-free survival (PFS) and overall survival were 4.6 months and 7.9 months, respectively. Univariate and multivariate analyses revealed that a tumor size >5 cm was associated with a worse prognosis (p = 0.045), while a radiation dose >58 GyEQD2 was significantly associated with better PFS (p = 0.038). @*Conclusion@#This study demonstrates clinical outcomes after radical radiotherapy in managing PSC, suggesting tumor size and radiation dose could be a predictor of a systemic response. Given the known bad prognosis but complete remission could be achieved in certain subgroups, future research should explore the potential strategies using radical radiotherapy for this challenging patient population.

Incidence and Risk Factors of Recurrent Venous Thromboembolism after Pulmonary Embolism / 결핵

BACKGROUND: Information about the epidemiology of venous thromboembolism (VTE) recurrence in Korea is lacking. The purpose of this study was to investigate VTE cumulative recurrence rates and identify risk factors for VTE recurrence among Korean adults. METHODS: A retrospective cohort study was conducted on adult patients (≥18 years) admitted to a university teaching hospital for pulmonary embolism (PE) from 2005 to 2013. The main outcome of interest was a recurrence of VTE. We used Cox proportional hazard regression analyses to calculate the relative risk of VTE recurrence. RESULTS: Five-year cumulative incidence of recurrent VTE events was 21.5% (95% confidence interval [CI], 17.7–25.4) in all cases of PE; 17% after provoked and 27% after unprovoked PE. Multivariate analysis showed that body mass index (BMI) of ≥25 (hazard ratio [HR], 2.02; 95% CI, 1.17–3.46; p=0.01) and longer anticoagulation therapy duration (HR, 0.90; 95% CI, 0.84–0.96; p<0.01) were independently associated with risk of VTE recurrence. Risk factors not found to be statistically significant at the <0.05 level included history of VTE (HR, 1.81; 95% CI, 0.84–3.88; p=0.12), unprovoked PE (HR, 1.70; 95% CI, 0.89–3.25; p=0.10), symptomatic deep vein thrombosis (HR, 1.62; 95% CI, 0.89–2.94; p=0.10), and female sex (HR, 1.42; 95% CI, 0.78–2.55; p=0.24). We found that age, history of cancer, and other co-morbidities did not significantly affect the risk of VTE recurrence. CONCLUSION: Recurrence of VTE after PE is high. Patients with BMI ≥25 or reduced anticoagulation therapy duration have a higher risk of recurrent VTE.

Cancer Survivors and Returning to Work Perspectives from Occupational Health Physicians in Korea

BACKGROUND: This study was conducted from an occupational health perspective to document cancer survivors' ability to return to work, the role of clinical care, and the current status of effective return-to-work. METHODS: This cross-sectional study was conducted to evaluate the experiences and opinions of occupational health physicians (OHPs) regarding cancer survivors' return-to-work. A self-reported survey was conducted from December 30, 2015, to January 30, 2016, targeting 337 OHPs. Questions included: 1) treatment experiences of survivors in the words of OHPs, 2) current status of the assessments of fitness for work of cancer survivors, 3) experiences associated with workplace and treatment, and 4) problems of returning to work and overcoming system. RESULTS: Only 25% of the respondents said that they had experience treating cancer survivors, and the average number of patients was 12.6 per annum, which indicated that few cancer survivors were treated. Eleven cases included conducting assessment of fitness for work. There were 17 respondents who did not treat cancer survivors. Both those who had and did not have experience in treating survivors showed higher musculoskeletal system disorders (53.8 vs. 63.5) than cancer (15.5 vs. 11.2) in terms of frequency of the diseases in the assessment of fitness for work. Most respondents said that OHPs evaluate the current role appropriately and preferred OHPs in the future. They responded that OHPs found it difficult to treat cancer survivors, and it was psychologically tough to communicate with them (61.4%). Regarding the association of patient rehabilitation with workplaces, 48.9% said that workplaces provide inadequate support. CONCLUSION: As a preliminary study, we found that OHPs were found to have little experience in treating cancer survivors and undergo difficulties owing to poor collaboration with workplaces and communication with patients. This study will provide basic data for future studies to promote cancer survivors' return to workplaces.

Higher Age Puts Lung Cancer Patients at Risk for Not Receiving Anti-cancer Treatment / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: We aimed to determine the demographic and epidemiologic variables that are associated with no treatment in lung cancer patients. MATERIALS AND METHODS: Patient data were collected from the Korean National Health Insurance Database. The lung cancer group included patients with an initial diagnosis of lung cancer between January 2009 and December 2014. Treated cases were defined as those that underwent surgery, radiation, or chemotherapy until death, after the diagnosis of lung cancer. Risk of no treatment was calculated by multiple logistic regression analysis. RESULTS: Among the 2,148 new cases of lung cancer from 2009 to 2104, 612 (28.4%) were not treated. Risk of no treatment was higher in the following patients: patients in their 60s (odds ratio [OR], 1.18; 95% confidence interval [CI], 0.75 to 1.84), 70s (OR, 3.64; 95% CI, 2.41 to 5.50), and >80 years old (OR, 16.55; 95% CI, 10.53 to 25.03) than those in their 50s; patients with previous myocardial infarction (OR, 2.07; 95% CI, 1.01 to 4.25) or chronic kidney disease (OR, 2.88; 95% CI, 1.57 to 5.30); and patients diagnosed at a non-referral hospital (OR, 1.40; 95% CI, 1.01 to 1.92) or primary care provider (OR, 1.81; 95% CI, 1.43 to 2.29) compared with referral hospital. Low-income patients receiving Medicaid were 1.75 times (95% CI, 1.14 to 2.68) more likely to forgo treatment than high-income patients (upper 20%). Risk was not associated with sex or the year in which the lung cancer was diagnosed. CONCLUSION: Age predominantly determines whether patients with lung cancer undergo anti-cancer treatment.

Incidence and Risk Factors of Recurrent Venous Thromboembolism after Pulmonary Embolism / 결핵및호흡기질환

BACKGROUND@#Information about the epidemiology of venous thromboembolism (VTE) recurrence in Korea is lacking. The purpose of this study was to investigate VTE cumulative recurrence rates and identify risk factors for VTE recurrence among Korean adults.@*METHODS@#A retrospective cohort study was conducted on adult patients (≥18 years) admitted to a university teaching hospital for pulmonary embolism (PE) from 2005 to 2013. The main outcome of interest was a recurrence of VTE. We used Cox proportional hazard regression analyses to calculate the relative risk of VTE recurrence.@*RESULTS@#Five-year cumulative incidence of recurrent VTE events was 21.5% (95% confidence interval [CI], 17.7–25.4) in all cases of PE; 17% after provoked and 27% after unprovoked PE. Multivariate analysis showed that body mass index (BMI) of ≥25 (hazard ratio [HR], 2.02; 95% CI, 1.17–3.46; p=0.01) and longer anticoagulation therapy duration (HR, 0.90; 95% CI, 0.84–0.96; p<0.01) were independently associated with risk of VTE recurrence. Risk factors not found to be statistically significant at the <0.05 level included history of VTE (HR, 1.81; 95% CI, 0.84–3.88; p=0.12), unprovoked PE (HR, 1.70; 95% CI, 0.89–3.25; p=0.10), symptomatic deep vein thrombosis (HR, 1.62; 95% CI, 0.89–2.94; p=0.10), and female sex (HR, 1.42; 95% CI, 0.78–2.55; p=0.24). We found that age, history of cancer, and other co-morbidities did not significantly affect the risk of VTE recurrence.@*CONCLUSION@#Recurrence of VTE after PE is high. Patients with BMI ≥25 or reduced anticoagulation therapy duration have a higher risk of recurrent VTE.

Interstitial Lung Disease and Lung Cancer Development: A 5-Year Nationwide Population-Based Study / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: In this nationwide 5-year longitudinal population-based study, we aimed at investigating the incidence of lung cancer among patients with interstitial lung disease. MATERIALS AND METHODS: Data was collected from the Korean National Health Insurance Research Database from 49,773,195 Korean residents in 2009. Thirteen thousand six hundred and sixty-six patients with interstitial lung disease diagnosed January-December 2009. The end of follow-up was June 30, 2014. Up to four matching chronic obstructive pulmonary disease controls were selected to compare the lung cancer high-risk group based on age, sex, diagnosis date (within 30 days), and hospital size. The number of patients with newly developed lung cancer was determined. RESULTS: The incidences of lung cancer were 126.98, 156.62, and 370.38 cases per 10,000 person-years (2,732, 809, and 967 cases of cancer, respectively) in the chronic obstructive pulmonary disease, interstitial lung disease, and chronic obstructive pulmonary disease with interstitial lung disease groups, respectively. Of the 879 patients with idiopathic pulmonary fibrosis, 112 developed lung cancer (incidence, 381.00 cases per 10,000 person-years). CONCLUSION: Incidence of lung cancer among patients with interstitial lung disease was high. Interstitial lung diseases have a high potential for developing into lung cancer, even when concurrent with chronic obstructive pulmonary disease.

A Case of Relapsing Polychondritis Associated with Ankylosing Spondylitis

Relapsing polychondritis is an uncommon disease associated with inflammation in cartilaginous tissues throughout the body, particularly affecting the cartilaginous structures of ears, nose, joints, and respiratory tract. Several autoimmune diseases, including vasculitis, are associated with the concurrent relapsing polychondritis. However, ankylosing spondylitis primarily affecting the sacroiliac joints and spine is rare in patients with relapsing polychondritis. We report on a 54-year-old man with concurrently relapsing polychondritis and ankylosing spondylitis.

Distal Renal Tubular Acidosis with Nephrocalcinosis in a Patient with Primary Sjogren's Syndrome

Renal involvement is not uncommon in primary Sjogren's syndrome; however, it is clinically insignificant in most cases. Distal renal tubular acidosis accounts particularly for the majority. While the underlying distal renal tubular acidosis is an important cause of nephrocalcinosis and urolithiasis, nephrocalcinosis is rarely a presenting feature of primary Sjogren's syndrome. We report a 65-year-old woman who was diagnosed with distal renal tubular acidosis accompanied by primary Sjogren's syndrome, according to nephrocalcinosis, which was incidentally identified by an abdominal ultrasonography during a medical examination.

A Case of Angiotensin Converting Enzyme Inhibitor-Induced Systemic Lupus Erythematosus

Drug-induced lupus erythematosus is defined as a lupus-like syndrome related to continuous drug exposure which resolves after discontinuation of the offending drug. Here we report a case of a 70-year-old man who developed drug-induced lupus erythematosus after receiving angiotensin converting enzyme inhibitor medication for unstable angina pectoris, for 5 years. He was hospitalized with arthralgia, edema, and newly developed pleural effusion. The serum analysis revealed an elevated level of antinuclear antibody and antihistone antibody. After discontinuation of angiotensin converting enzyme inhibitor and receiving a course of prednisolone treatment, his symptoms and pleural effusion improved. To the best of our knowledge, this is, the first case report of angiotensin converting enzyme inhibitor-induced systemic lupus erythematosus in Korea.

Microscopic Polyangiitis Presenting as Membranoproliferative Glomerulonephritis / 대한류마티스학회지

Microscopic polyangiitis (MPA) is characterized by pauci-immune necrotizing small vessel vasculitis without clinical or pathological evidence of necrotizing granulomatous inflammation. The kidney is the most often affected organ in the majority of patients with MPA, and renal manifestations are usually the first symptoms. Glomerular capillaries are affected most often, resulting in necrotizing glomerulonephritis, usually in a crescent formation, with no or few immune deposits able to be demonstrated at the sites of vasculitis and glomerulonephritis. We report a case of microscopic polyangiitis in both legs with pitting edema in a 50-year-old female. Laboratory findings showed hematuria, proteinuria, and a positive peripheral antineutrophil cytoplasmic antibody. A renal biopsy revealed pauci-immune splitting and necrotizing capillary loop walls necrotizing vasculitis and membranoproliferative glomerulonephritis (MPGN). With a diagnosis of MPA, she has been managed with high dose steroid and cyclophosphamide. To our knowledge, this is the first reported case of MPA with MPGN.

A Case of CPPD Crystal Deposition Disease in a Patient with Rheumatoid Arthritis and Systemic Sclerosis / 대한류마티스학회지

Calcium pyrophosphate dihydrate (CPPD) crystal deposition disease is an inflammatory arthropathy that is defined by the deposition of CPPD crystals in articular and periarticular structures. CPPD crystal deposition disease has various clinical manifestation patterns ranging from an absence of symptoms to a severely destructive arthropathy. CPPD crystal deposition disease very rare with rheumatoid arthritis or systemic sclerosis. We report a case of CPPD crystal deposition disease combined in a patient with rheumatoid arthritis and systemic sclerosis.

A Case of Ankylosing Spondylitis in a Patient with Human Immunodeficiency Virus / 대한류마티스학회지

Human immunodeficiency virus infection is associated with a variety of rheumatic manifestations. The pathogenic mechanisms are not well defined, but evidences suggests multiple mechanisms, including direct or indirect human immunodeficiency virus involvement with a genetic, immunological, and environmental basis. Rheumatic manifestations can occur during the course of human immunodeficiency virus infection, and their presence is associated with an overall poor prognosis for the underlying human immunodeficiency virus infection. We report a case of a 37-year-old male who had ankylosing spondylitis with a human immunodeficiency virus infection.

A case of essential thrombocythemia in a patient with Behcet's disease / 대한내과학회지

Behcet's disease (BD) is a chronic inflammatory disorder characterized by vasculitis of unknown cause and involving multiple organs. Its pathogenesis includes neutrophil hyperfunction and the overproduction of inflammatory cytokines, including INF.alpha. BD is often accompanied by leukocytosis, but is rarely associated with myeloproliferative disease. Essential thrombocythemia (ET) is a myeloproliferative disorder characterized by marked thrombocytosis and marrow megakaryocytic hyperplasia. Only one case of ET associated with incomplete.type intestinal BD during hydroxyurea treatment has been reported. Here, we report a case of essential thrombocythemia in 53.year.old female with BD who had taken no medication. Based on the history, physical examination, and routine laboratory and bone marrow examination, we diagnosed her with ET.

Pulse wave velocity in young premenopausal women with systemic lupus erythematosus / 대한내과학회지

BACKGROUND/AIMS: We measured the brachial artery pulse wave velocity (BaPWV) and intima-media thickness (IMT) of the common carotid artery to evaluate atherosclerosis in young women with systemic lupus erythematosus (SLE) and healthy controls. METHODS: Twenty-one premenopausal female patients with SLE and 21 healthy controls were enrolled. The groups were matched for age, sex, ethnicity, and atherosclerosis risk factors. Smokers and those with the atherosclerosis risk factors hypertension and diabetes were excluded from this study. IMT was measured with high-resolution ultrasonography. BaPWV was measured with volume plethysmography. RESULTS: The mean age was 34.20 years in the SLE group and 36.87 years in the healthy controls. The IMT of the right and left common carotid arteries in the SLE group did not differ significantly from that in the healthy controls. The BaPWV was significantly higher in the right (1.40+/-0.26 vs. 1.17+/-0.09 cm/sec, mean+/-SD; p=0.003) and left (1.41+/-0.26 vs. 1.19+/-0.08 cm/sec, p=0.013) arms in the SLE group than in the healthy controls. There were no significant differences in the ankle-brachial pressure index (ABI), body mass index (BMI), C-reactive protein (CRP), total cholesterol, high-density lipoprotein (HDL) cholesterol, low-density lipoprotein (LDL) cholesterol, lipoprotein a, and homocysteine between the two groups. CONCLUSIONS: The IMT in premenopausal women with SLE was not significantly different from that in healthy controls, while the BaPWV, which reflects arterial stiffness, was significantly higher than in the healthy controls.

A Case of Ankylosing Spondylitis with Cricoarytenoid Arthritis / 대한류마티스학회지

The cricoarytenoid joint is a diathrodial synovial joint and it can be affected by various diseases. The etiology includes infectious, rheumatic and degenerative diseases. Cricoarytenoid arthritis that's caused rheumatoid arthritis is the most well known, yet cricoarytenoid arthritis that's caused by ankylosing spondylitis is very rare. Hoarseness, dysphagia, throat discomfort, dyspnea and dyspnea on exertion are the major symptoms. If there was no severe airway obstruction, this condition can be managed by systemic steroid or intraarticular steroid injection. We experienced a case of a 47 years old man with ankylosing spondylitis and he presented with hoarseness, dyspnea on exertion and dysphagia. He was finally diagnosed with cricoarytenoid arthritis and he improved after etanercept administration. So we report here on this case along with a review of relevant literature.

A case of acute myocardial infarction associated with coronary vasospasm in a patient with systemic sclerosis / 대한내과학회지

Systemic sclerosis is a chronic, systemic disease that targets the skin, lungs, heart, gastrointestinal tract, kidneys, and musculoskeletal system. Prominent features are vascular damage, immune activation, and the deposition of extravascular matrix. Scleroderma heart disease manifests as myocardial fibrosis. Intermittent spasm of the blood vessels in patients with systemic sclerosis may result in contraction band necrosis, similar to the changes observed in myocardial infarction in patients with atherosclerotic coronary artery disease. Coronary vasospasm has been demonstrated during attacks of cold-induced Raynaud's disease. However, acute myocardial infarction associated with coronary vasospasm in patients with systemic sclerosis has not been reported. We describe a rare case of acute myocardial infarction due to coronary vasospasm associated with systemic sclerosis in a 43-year-old woman who presented with severe chest pain.

RANKL stimulates proliferation, adhesion and IL-7 expression of thymic epithelial cells

Abstract In many clinical situations which cause thymic involution and thereby result in immune deficiency, T cells are the most often affected, leading to a prolonged deficiency of T cells. Since only the thymic-dependent T cell production pathway secures stable regeneration of fully mature T cells, seeking strategies to enhance thymic regeneration should be a key step in developing therapeutic methods for the treatment of these significant clinical problems. This study clearly shows that receptor activator of NF-kappaB ligand (RANKL) stimulates mouse thymic epithelial cell activities including cell proliferation, thymocyte adhesion to thymic epithelial cells, and the expression of cell death regulatory genes favoring cell survival, cell adhesion molecules such as ICAM-1 and VCAM-1, and thymopoietic factors including IL-7. Importantly, RANKL exhibited a significant capability to facilitate thymic regeneration in mice. In addition, this study demonstrates that RANKL acts directly on the thymus to activate thymus regeneration regardless of its potential influences on thymic regeneration through an indirect or systemic effect. In light of this, the present study provides a greater insight into the development of novel therapeutic strategies for effective thymus repopulation using RANKL in the design of therapies for many clinical conditions in which immune reconstitution is required.

Replacement of Obstructed Extracardiac Conduits with Autologous Tissue Reconstructions (Peel operation); Early and Midterm Results / 대한흉부외과학회지

BACKGROUND: Reoperation is usually required for a right ventricle to pulmonary artery conduit obstruction caused by valve degeneration, conduit peel formation or somatic growth of the patient. An autologous tissue reconstruction (peel operation), where a prosthetic roof is placed over the fibrotic tissue bed of the explanted conduit, has been used to manage conduit obstructions at our institute since May 2002. Herein, the early and midterm results are evaluated. MATERIAL AND METHOD: Between May 2002 and July 2006, 9 patients underwent obstructed extracardiac conduit replacement with an autologous tissue reconstruction, at a mean of 5.1 years after a Rastelli operation. The mean age at reoperation was 7.5+/-2.4 years, ranging from 2.9 to 10.1 years. The diagnoses included 6 pulmonary atresia with VSD, 2 truncus arteriosus and 1 transposition of the great arteries. The preoperative mean systolic gradient was 88.3+/-22.2 mmHg, ranging from 58 to 125 mmHg. The explanted conduits were all Polystan valved pulmonary conduit (Polystan, Denmark). A bioprosthetic valve was inserted in 8 patients, and a monocusp ventricular outflow patch (MVOP) was used in 1 patient. The anterior wall was constructed with a Gore-Tex patch (n=7), MVOP (n=1) and bovine pericardium (n=1). Pulmonary artery angioplasty was required in 5 patients and anterior aortopexy in 2. The mean cardiopulmonary bypass time was 154 minutes, ranging from 133 to 181 minutes; an aortic crossclamp was not performed in all patients. The mean follow-up duration was 20 months, ranging from 1 to 51 months. All patients were evaluated for their right ventricular outflow pathway using a 3-D CT scan. RESULT: There was no operative mortality or late death. The mean pressure gradient, assessed by echocardiography through the right ventricular outflow tract, was 20.4 mmHg, ranging from 0 to 29.6 mmHg, at discharge and 26 mmHg, ranging from 13 to 36 mmHg, at the latest follow-up (n=7, follow-up duration >1 year). There were no pseudoaneurysms, strictures or thrombotic occlusions. CONCLUSION: A peel operation was concluded to be a safe and effective re-operative option for an obstructed extracardiac conduit following a Rastelli operation.

Manifestations Like Multiple Sclerosis in a Paitent with Rheumatoid Arthritis and Sjogren's Syndrome / 대한류마티스학회지

Demyelinating syndrome can rarely occur in Sjogren's syndrome or rheumatoid arthritis. We describe a patient of Sjogren's syndrome with multiple sclerosis-like features whose rheumatoid arthritis has been managed for 3 years. The patient presented paraparesis and urinary retention, and improved with high-dose corticosteroid therapy.

Pulmonary hypertension in systemic lupus erythematosus / 대한내과학회지

BACKGROUND: Pulmonary arterial hypertension (PAH) in systemic lupus erythematosus (SLE) has very poor prognosis and the prevalence of the disease is also not well delineated. This study was designed to assess the prevalence of PAH in asymptomatic SLE patients and to analyze the potential association between the presence of PAH and SLE including disease activity, organ involvement, and serology. METHODS: Asymptomatic 50 SLE patients and 50 healthy controls of a similar age and sex were assessed by an echocardiographic and clinical study. The diagnosis of PAH was made when the right ventricular systolic pressure (RVSP), measured by 2-dimensional echocardiography, was > or =40 mmHg. RESULTS: Compared to control subjects, lupus patients had significantly increased RVSP (mean+/-SD, 34.9+/-8.9 vs 31.2+/-3.6 mmHg, p=0.008). Patients with SLE were divided into three groups: RVSP> or =40 mmHg, RVSP=30~39 mmHg, RVSP or =40 mmHg, 36 patients (72%) had RVSP=30~39 mmHg and 7 patients (14%) had RVSP or =40 mm Hg. Of the controls, 43 (86%) had RVSP=30~39 mmHg and 7 (14%) had RVSP<30 mmHg. There was no statistical difference in organ involvement, Raynaud's phenomenon, vasculitis, anti-RNP and anti-cardiolipin Ab among the three SLE groups. However, lupus disease activity (checked by SLEDAI-2K) was associated with PAH (p=0.014). CONCLUSIONS: The prevalence of PAH as seen on echocardiograms in SLE patients was 14%. Organ involvement, Raynaud's phenomenon, vasculitis, antiphopholipid antibodies and anti-RNP except SLEDAI-2K were not associated with pulmonary arterial pressure.