Pulmonary nodules and primary Sjögren syndrome: a case report.

Primary Sjögren syndrome (pSS) is a systemic autoimmune disorder that principally affects the exocrine glands but can also affect systemic or extra-glandular sites. Approximately 65-80% of patients with Sjogren's demonstrate pulmonary involvement at the CT scan and pulmonary nodules (PNs) can be encountered as a common finding. We present the case of a 49-year-old woman admitted to the emergency department for chest pain and fever. The patient was diagnosed with pSS fourteen years prior and had never taken therapy or followed regular check-ups. At the HRTC were found PNs that were studied trough a CT-PET and a needle biopsy via CT guidance, which showed diffuse large B cell lymphoma. This case report underlies the importance of check-ups and the need for a multidisciplinary approach in the care of Sjögren's syndrome patients.

DIPNECH: pragmatic approach, uncertainties, notable associations, and a proposal for an improved definition.

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare, but increasingly recognized entity that primarily affects middle-aged and elderly women. It is characterized by abnormal proliferation of pulmonary neuroendocrine cells (PNECs) and is considered a preinvasive lesion for carcinoid tumorlets/tumors. Sometimes, DIPNECH is accompanied by constrictive bronchiolitis which usually manifests as chronic cough and/or dyspnea, along with airflow limitation on spirometry. The telltale imaging sign of DIPNECH is the presence of multiple noncalcified pulmonary nodules and mosaic attenuation on CT. However, these clinico-radiologic features of DIPNECH are characteristic but nonspecific; thus, histopathologic confirmation is usually necessary. DIPNECH has an indolent course and only rarely leads to respiratory failure or death; progression to overt neuroendocrine tumor (carcinoid) of the lung occurs in a minority of patients. Of available therapies, somatostatin analogs and mechanistic target of rapamycin inhibitors are the most promising. In this review, we provide an update regarding the diagnosis and management of DIPNECH and describe critical gaps in our understanding of this entity, including the central terms 'diffuse' and 'idiopathic.' We also summarize the inconsistencies in definitions employed by recent studies and discuss the pitfalls of the DIPNECH definitions proposed by the World Health Organization in 2021. In this context, we propose an objective and reproducible radio-pathologic case definition intended for implementation in the research realm and seeks to enhance homogeneity across cohorts. Furthermore, we discuss aspects of PNECs biology which suggest that PNEC hyperplasia may contribute to the pathogenesis of phenotypes of lung disease aside from constrictive bronchiolitis and carcinoid tumorlets/tumors. Finally, we steer attention to some of the most pressing and impactful research questions awaiting to be unraveled.

Comparison of three-dimensional reconstruction and CT-guided Hook-wire segmental resection for pulmonary nodules: a propensity score matching study.

OBJECTIVE: To analyze and compare the clinical application value of three-dimensional reconstruction and computed tomography (CT)-guided Hook-wire localization for row lung segment resection of pulmonary nodules. METHODS: Retrospective analysis of the clinical data of 204 patients suffering from pulmonary nodules admitted to the Department of Thoracic Surgery of Gansu Provincial People's Hospital from June 2016 to December 2022. According to the preoperative positioning method, the group was divided into a 3D reconstruction group (98 cases) and a Hook-wire group (106 cases), respectively. The two groups of patients were propensity score matching (PSM) to compare their perioperative outcomes. RESULTS: All patients in both groups underwent successful surgeries without perioperative deaths. After PSM, 79 patients were successfully matched in each group. Two cases of pneumothorax, three cases of hemothorax, and four cases of decoupling occurred in the Hook-wire group; no complications of pneumothorax, hemothorax, and decoupling occurred in the 3D reconstruction group. Compared to the Hook-wire group, the 3D reconstruction group has shorter operative time (P = 0.001), less intraoperative bleeding (P < 0.001), less total postoperative chest drainage (P = 0.003), shorter postoperative tube placement time (P = 0.001), shorter postoperative hospital stay (P = 0.026), and postoperative complications (P = 0.035). There was no statistically significant difference between the two groups in terms of pathological type, TNM staging, and number of lymph node dissection. CONCLUSION: Three-dimensional reconstruction and localization of pulmonary nodules enables safe and effective individualized thoracoscopic anatomical lung segment resection with a low complication rate, which has good clinical application value.

Diagnostic follow-up of indeterminate pulmonary nodules in the Medicare population.

BACKGROUND: Management of indeterminate pulmonary nodules (IPNs) is associated with redistribution of lung cancer to earlier stages, but most subjects with IPNs do not have lung cancer. The burden of IPN management in Medicare recipients was assessed. METHODS: Surveillance, Epidemiology, and End Results-Medicare data were analyzed for IPNs, diagnostic procedures, and lung cancer status. IPNs were defined as chest computed tomography (CT) scans with accompanying International Classification of Diseases (ICD) codes of 793.11 (ICD-9) or R91.1 (ICD-10). Two cohorts were defined: persons with IPNs during 2014-2017 comprised the IPN cohort, whereas those with chest CT scans without IPNs during 2014-2017 comprised the control cohort. Excess rates of various procedures due to reported IPNs over 2 years of follow-up (chest CT, positron emission tomography [PET]/PET-CT, bronchoscopy, needle biopsy, and surgical procedures) were estimated using multivariable Poisson regression models comparing the cohorts adjusted for covariates. Prior data on stage redistribution associated with IPN management were then used to define a metric of excess procedures per late-stage case avoided. RESULTS: Totals of 19,009 and 60,985 subjects were included in the IPN and control cohorts, respectively; 3.6% and 0.8% had lung cancer during follow-up. Excess procedures per 100 persons with IPNs over a 2-year follow-up were 63, 8.2, 1.4, 1.9, and 0.9 for chest CT, PET/PET-CT, bronchoscopy, needle biopsy, and surgery, respectively. Corresponding excess procedures per late-stage case avoided were 48, 6.3, 1.1, 1.5, and 0.7 based on an estimated 1.3 late-stage cases avoided per 100 IPN cohort subjects. CONCLUSIONS: The metric of excess procedures per late-stage case avoided can be used to measure the benefits-to-harms tradeoff of IPN management.

Syphilis With Multiple Pulmonary Nodules and Bone Lesions Detected on Computed Tomography and 18F Fluorodeoxyglucose-Positron Emission Tomography/Computed Tomography.

Syphilis can cause a wide range of systemic manifestations, such as papular rash, malaise, weight loss, muscle aches, generalized lymphadenopathy, and meningitis. However, pulmonary involvement in patients with secondary syphilis is thought to be relatively rare. Moreover, bone involvement in patients with secondary syphilis is also considered rare, and only a few cases of involvement of lung and bone in such patients have been reported. In this paper, we report a case of secondary syphilis with pulmonary involvement in the form of multiple nodules with low attenuation areas, lymphadenopathy and multiple bone lesions detected on computed tomography and 18F fluorodeoxyglucose-positron emission tomography/computed tomography.

Large Thoracic Lymphadenopathy and Pulmonary Nodules in Young Man.

CASE PRESENTATION: A 33-year-old man with obesity, systemic arterial hypertension, and psoriasis who had been treated previously with little success by a pulmonologist for chronic unproductive irritant cough came to the outpatient pulmonary department because of profuse cough and short syncope (probably cough-induced). Chest radiography revealed widened mediastinum with lobular, polycyclic contours that was suspected to be a large mediastinal lymphadenopathy or mediastinal mass.

A Liposarcoma Arising in a Pulmonary Hamartoma, Coexisting With Benign Metastasizing Leiomyoma.

Pulmonary hamartoma is a common benign tumor that rarely degenerates into malignancy. This report documents a unique case of pulmonary hamartoma with malignant transformation into well-differentiated liposarcoma, coexisting in proximity to pulmonary nodules representing benign metastasizing leiomyoma in a 60-year-old woman.

Shortness of Breath in a 38-Year-Old Woman With Pulmonary and Hepatic Nodules.

CASE PRESENTATION: A 38-year-old African American woman with a history of menometrorrhagia on previous estrogen therapy and a previously biopsied benign thyroid nodule with recent interval enlargement presented with symptoms of shortness of breath on exertion, an intermittent nonproductive cough, and right upper quadrant abdominal pain for 1 year. She denied wheezing, hemoptysis, fevers, night sweats, or unintentional weight loss. Socially, the patient was a lifelong nonsmoker and denied alcohol or drug use. Travel history was not significant, and she had no contributory occupational, environmental, or animal exposures. Recent cancer screening that included Papanicolaou smear and mammography were negative for neoplasia. Vital signs were normal, and ambulatory pulse oximetry did not demonstrate evidence of oxygen desaturation. Physical examination demonstrated normal respiratory effort, diffuse vesicular breath sounds, and a soft abdomen without hepatomegaly or right upper quadrant tenderness.

Management of Pulmonary Nodules in Oncologic Patients: AJR Expert Panel Narrative Review.

Cancer survivors are at higher risk than the general population for development of a new primary malignancy, most commonly lung cancer. Current lung cancer screening guidelines recommend low-dose chest CT for high-risk individuals, including patients with a history of cancer and a qualifying smoking history. However, major lung cancer screening trials have inconsistently included cancer survivors, and few studies have assessed management of lung nodules in this population. This narrative review highlights relevant literature and provides expert opinion for management of pulmonary nodules detected incidentally or by screening in oncologic patients. In patients with previously treated lung cancer, a new nodule most likely represents distant metastasis from the initial lung cancer or a second primary lung cancer; CT features such as nodule size and composition should guide decisions regarding biopsy, PET/CT, and CT surveillance. In patients with extrapulmonary cancers, nodule management requires individualized risk assessment; smoking is associated with increased odds of primary lung cancer, whereas specific primary cancer types are associated with increased odds of pulmonary metastasis. Nonneoplastic causes, such as infection, medication toxicity, and postradiation or postsurgical change, should also be considered. Future prospective studies are warranted to provide evidence-based data to assist clinical decision-making in this context.

A 47-Year-Old Woman With Pulmonary Nodules and Facial Hemispasms.

CASE PRESENTATION: A 47-year-old woman visited her primary physician for a health check, and some radiographic abnormalities were detected. She was referred to our division for further management. In recent years, she had become conscious of occasional facial hemispasms. She denied respiratory symptoms, smoking, alcohol consumption, and any particle inhalation. She had undergone oophorectomy and platinum-based chemotherapy because of ovarian cancer (serous cystadenocarcinoma stage Ⅰa) at the age of 29 years, with no recurrence for 17 years. The patient was diagnosed with rheumatoid arthritis (RA) 5 years before being seen by us and had been treated with bucillamine. No signs of RA progression were evident, and the only used antirheumatic drug was bucillamine. The patient had no history of use of immune-modulating drugs or immunosuppressants. No previous chest radiographs or CT had been performed.

Cost-effectiveness of a new autoantibody test added to Computed Tomography (CT) compared to CT surveillance alone in the diagnosis of lung cancer amongst patients with indeterminate pulmonary nodules.

Oncimmune's EarlyCDT®-Lung is a simple ELISA blood test that measures seven lung cancer specific autoantibodies and is used in the assessment of malignancy risk in patients with indeterminate pulmonary nodules (IPNs). The objective of this study was to examine the cost-effectiveness of EarlyCDT-Lung in the diagnosis of lung cancer amongst patients with IPNs in addition to CT surveillance, compared to CT surveillance alone which is the current recommendation by the British Thoracic Society guidelines. A model consisting of a combination of a decision tree and Markov model was developed using the outcome measure of the quality adjusted life year (QALY). A life-time time horizon was adopted. The model was parameterized using a range of secondary sources. At £70 per test, EarlyCDT-Lung and CT surveillance was found to be cost-effective compared to CT surveillance alone with an incremental cost-effectiveness ratio (ICER) of less than £2,500 depending on the test accuracy parameters used. It was also found that EarlyCDT-Lung can be priced up to £1,177 and still be cost-effective based on cost-effectiveness acceptance threshold of £20,000 / QALY. Further research to resolve parameter uncertainty, was not found to be of value. The results here demonstrate that at £70 per test the EarlyCDT-Lung will have a positive impact on patient outcomes and coupled with CT surveillance is a cost-effective approach to the management of patients with IPNs. The conclusions drawn from this analysis are robust to realistic variation in the parameters used in the model.

Colorectal cancer with indeterminate pulmonary nodules.

Colorectal cancer (CRC) is one of the most common malignancies with a dismal prognosis. Indeterminate pulmonary nodules (IPNs) are lung nodules with uncertain nature, generally defined as a noncalcified nodule smaller than 10 mm in diameter or solid nodule no greater than 20 mm at maximum diameter without malignant character. With the widespread use of preoperative staging computed tomography (CT) of the chest and follow-up CT, IPNs are frequently detected in patients with CRC, which makes diagnosis more controversial. Generally, progression to pulmonary metastasis from IPNs is rare. Thus, no further interventions were needed for IPNs in CRC patients. A second reviewing of scans with IPNs by both clinicians and experienced thoracic radiologists may help to obtain a more accurate diagnosis.

A 45-Year-Old Woman With Multiple Pulmonary Nodules and Sjögren Syndrome.

CASE PRESENTATION: A 45-year-old woman presented for evaluation for 3 months of coughing and dyspnea. A recent chest CT scan done for workup of her symptoms revealed a 2-cm right-sided pulmonary nodule. She had a medical history of Sjögren syndrome, hypertension, and obesity. She also noted a weight loss of 30 lb over the last 3 years. She denied smoking, alcohol consumption, illicit drug use, or occupational exposures. A chest radiograph done 3 years prior did not reveal any pulmonary nodules. She had no personal or family history of arteriovenous malformations, hamartomas, or any malignancies and had been up to date with her breast cancer screening. She was treated with courses of hydroxychloroquine and mycophenolate mofetil for her Sjögren syndrome and did not have a history of opportunistic pulmonary infections. She denied any recent travel or exposure to TB.

A 31-Year-Old Man With Airflow Obstruction, Intrathoracic Adenopathy, and Pulmonary Nodules.

CASE PRESENTATION: A 31-year-old white man presented for evaluation of productive cough and dyspnea on exertion of 2 months' duration. Associated symptoms included wheezing, chest tightness, and postnasal drip. He was recently treated for pneumonia with a 7-day course of levofloxacin because of an abnormal chest radiograph demonstrating bilateral infiltrates, but his symptoms failed to improve. He had a medical history of mild intermittent asthma and was on no active treatment. He was a nonsmoker. He was born in the United States. He denied any recent travel within or outside the United States, sick contacts, or illicit drug use. He did not have any pets and denied exposure to mold, hot tubs, a down comforter, or pillows. He worked in a vocational program and his hobbies included refinishing wood floors.

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: Report of two cases.

INTRODUCTION: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare disorder characterized by a proliferation of neuroendocrine cells within the lung. It is classically described as a disease with persistent cough, dyspnea and wheezing in non-smoker middle aged females. CT of the chest reveals diffuse air trapping with mosaic pattern. PATIENTS AND METHODS: We present two cases of DIPNECH that were sent to our department to perform a lung biopsy with the diagnostic suspicion of diffuse interstitial disease. Both cases were women with a history of chronic cough and moderate effort dyspnea. RESULTS AND DISCUSSION: The aim of this paper is that physicians take into account this diagnostic entity before treating as an asthmatic a patient with these characteristics, not forgetting that they are prenoplastic lesions.

Management of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: Review and a Single Center Experience.

BACKGROUND: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary condition, characterized by diffuse proliferation of neuroendocrine cells in the respiratory epithelium. DIPNECH lesions are less than 5 mm in size and are limited to the basement membrane with no invasion. There is limited information regarding epidemiology, natural history of disease progression, or the management of this rare entity. We present the experience of a center with extensive expertise in neuroendocrine disease. METHODS: A cohort of patients (N = 13) with DIPNECH treated and followed at our institution was identified. We describe the our approach to their care, our disease management and also provide a review of DIPNECH pathophysiology. RESULTS: Our patient cohort consisted of twelve females and one male with a mean age of 63 years at the time of diagnosis. Dyspnea on exertion and dry cough were the most common presenting symptoms. Two patients were under surveillance without treatment; three patients were treated with a short-acting somatostatin analog; three patients were treated with azithromycin alone; four were treated with a combination of long-acting monthly somatostatin analogs and azithromycin; one patient received a combination of long-acting somatostatin analog and everolimus. Five patients had concomitant bronchial carcinoids. CONCLUSIONS: DIPNECH is a rare pathology that can profoundly affect a patient's quality of life. Paroxysmal coughing episodes can be difficult to treat. Our limited single center experience shows encouraging response to use of somatostatin analogs, azithromycin, and everolimus in the management of debilitating DIPNECH associated symptoms.