Mycoplasma pneumoniae-related hepatitis in children.

Mycoplasma pneumoniae (M. pneumoniae) is a small bacterium characterized by the absence of cell wall. It is a human pathogen causing upper and lower respiratory infections, both in adults and children. However, it is also considered to be implicated in the pathogenesis of several types of extra-respiratory diseases, including some gastrointestinal disorders. The liver involvement in children during or after M. pneumoniae infections is analyzed and discussed in this review. Through a systematic literature search, it is evidenced that M. pneumoniae is not infrequently associated with alteration of liver function, but rarely causes acute and severe hepatitis in children. M. pneumoniae should be considered as an unusual cause of acute hepatitis in children, whenever the most common hepatotropic viruses have been excluded. The pathogenesis of M. pneumoniae-related hepatitis is likely to be immune-mediated: both the innate and adaptive immune responses may play a fundamental role. However, the exact pathological mechanisms have to be elucidated yet. Further clinical studies are needed in order to understand the actual relevance of this microorganism in liver disease and its pathogenesis.

[Pulmonary manifestations of crohn's disease or chronic pharmacotherapy complications? - case report].

Pareneteral manifestations of Crohn's disease (ChLC), apart from the most common skin and joint symptoms include also complications from the respiratory system. In addition chronic pharmacotherapy of ChLC, especially 5-aminosalicylic acid or anti-TNF- α drugs, is associated with possible pulmonologic side effects, sometimes difficult to differentiate. In this study, we describe a patient with ChLC, with a history of pneumocystic pneumonia, who was diagnosed with exfoliative institial pneumonitis as a result of chronic use of mesalazine. This disease is characterized by accumulation of alveolar macrophages in the lumen of the alveoli and intrabepticular septum. The most common etiologic factor is exposure to tobacco smoke. Our patient, non-smoker, was finally diagnosed after lung biopsy and histopathological evaluation. The gradual clinical improvement after mesalazine was an additional factor confirming the etiology of the disease. This side effect of mesalazine is not common but it should be considered in all patient treated with 5-aminosalicylic acid.

IgG4-related respiratory disease.

IgG4-related diseases (IgG4-RDs), such as autoimmune pancreatitis and IgG4-related Mikulicz disease, are often accompanied by intrathoracic lesions, which are called IgG4-related respiratory disease (IgG4-RRD). IgG4-RRD has few subjective symptoms, and is usually detected during workup of patients with extra-thoracic lesions of IgG4-RD. IgG4-RRD is characterized by various conditions, including masses, nodules, thickening, and infiltration at numerous sites in the thorax through lymphatic routes. Although elevated serum IgG4 concentrations and pathologic evidence of lymphoplasmacytic infiltrates with abundant IgG4-positive plasma cells are characteristic findings of IgG4-RD, other intrathoracic diseases, such as multicentric Castleman disease and malignancy, may present with similar findings. Developing diagnostic criteria for IgG4-RRD, including clinicoradiological and pathological characteristics, is necessary for its appropriate diagnosis.

[Aspects of pulmonary involvement in inflammatory bowel disease]. / Lungenbeteiligung bei chronisch-entzündlichen Darmerkrankungen.

The incidence of pulmonary manifestations of inflammatory bowel disease (IBD) appears to be much higher than previously assumed. In prospective studies, subclinical pulmonary interstitial infiltrates or pathological lung function were found in 40%-60% of IBD patients, both in children and adults. Pulmonary disorders can affect any part of the respiratory system, the most frequent pattern being inflammation of the large airways often associated with bronchiectasis. The differential diagnosis should include drug-related pulmonary disease and infectious causes when receiving immunosuppressive therapy. The diagnostic approach consists of a thorough history and clinical status as well as lung function tests including body plethysmography and high-resolution computed tomography of the thorax. Bronchoscopy with broncheoalveolar lavage and sample collection for histology as well as exclusion of pulmonary embolism may be indicated. Pulmonary disease in association with IBD can develop at any time during the course of IBD: in rare cases, symptoms can evolve even before gastrointestinal symptoms appear. On the other hand, there are frequent reports on the occurrence of pulmonary inflammation after proctocolectomy in patients with ulcerative colitis. The pathophysiologic background is largely unknown, but there seems to be an interaction between gastrointestinal and pulmonary inflammation. The mainstay of therapy are inhaled or systemic corticosteroids. Most patterns of pulmonary involvement in IBD respond well to corticosteroid therapy. Rarely, serious and persisting complications occur, such as strictures or stenosis of the large airways.

Pulmonary manifestations of systemic lupus erythematosus patients with and without antiphospholipid syndrome.

OBJECTIVE: To uncover the pulmonary manifestations of Systemic Lupus Erythematosus (SLE) patients alone and to compare findings with antiphospholipid syndrome (APS) associated with SLE. METHODS: This cross sectional comparative study was carried out at King Khalid University Hospital (KKUH)/King Saud University (KSU), a tertiary care hospital, Riyadh, Kingdom of Saudi Arabia. From June 2012 to March 2014, 96 diagnosed SLE patients with respiratory symptoms were included in the study and divided into two groups. Group one included SLE without antiphospholipid syndrome (APS) and group two SLE with APS. We compared Demographic features, clinical manifestations and findings of chest X-Ray, Arterial Blood Gases, Pulmonary function tests, six minute walk test, ventilation perfusion scan, echocardiography and chest high resolution computed tomography. RESULTS: Demographic and clinical characteristics of two groups were similar. Previous history of deep venous thrombosis (3% vs 27.6%, p=0.001), pulmonary embolism (3% vs34.5%, p<0.0001) and abortions (7.5% vs 27.6%, p=0.019) were significantly more in group two. Levels of Anticardiolipin antibody (0% vs 100%, p<0.0001) and lupus anticoagulant (1.5% vs 79.3%, p<0.0001) were also significantly higher in group two. Hypoxemia measured by pulse oximetry (43.3% vs 65.5% p=0.045, pulmonary Arterial Hypertension (15.5% vs 39.3% p=0.014)), and pulmonary embolism (3.4% vs 21.4% p=0.013) and ventilation perfusion mismatch on V/Q scan (1.5% vs 24.1% p=0.001) were more frequent in group two. CONCLUSION: Hypoxemia, pulmonary embolism and pulmonary arterial hypertension were significantly high in SLE patients with APS, requiring long term anticoagulation and treatment and close follow-up.

Respiratory Aspects of Primary Ciliary Dyskinesia.

This review article explores the respiratory aspects of primary ciliary dyskinesia (PCD), a rare, heterogenous, genetic disorder characterized by impaired motile ciliary function. It discusses the clinical diagnosis and management strategies for PCD-related respiratory disease, including chronic sinusitis, otitis media with effusion, recurrent pneumonia, and bronchiectasis. The review emphasizes the need for a multidisciplinary approach to optimize care and clinical trials to improve outcomes in individuals with PCD, highlighting the importance of accurate diagnosis.

Pulmonary Manifestations of IBD: Case Report and Review of the Literature.

This article explores the pulmonary complications associated with inflammatory bowel disease (IBD). It presents a detailed case study of a 22-year-old male with Crohn's disease exhibiting pulmonary symptoms. The review delves into the spectrum of pulmonary involvement in IBD, covering clinical presentations, diagnostic challenges, underlying pathophysiology, and management strategies. It highlights the significance of these extraintestinal manifestations on patient outcomes and quality of life. The article underscores the need for heightened clinical awareness and a systematic approach to diagnosis and management, integrating the expertise of multiple specialists. The review identifies gaps in current research, suggesting avenues for future investigation to enhance the understanding and treatment of these complex manifestations.

Extra-pulmonary Cutaneous Sarcoidosis Presenting With Granulomatous Cranial Lesions and Cardiac Complications: A Case Report.

Sarcoidosis is a non-caseating granulomatous disorder affecting multiple organs. Although the lungs are the most common site of presentation, extra-pulmonary manifestations involving the skin and heart can occur. Sarcoidosis affecting skull bone is uncommon and involvement of skin, heart, and skull bone all together, without pulmonary manifestations, is extremely rare. We report a 63-year-old Caucasian woman with a past history of cutaneous sarcoidosis and granulomatous skull bone lesions who presented with recurrent syncope. An ambulatory cardiac monitor detected intermittent high-grade atrioventricular block and cardiac MRI confirmed the diagnosis of cardiac sarcoidosis. This case represents an extremely unique journey of sarcoidosis and suggests potential consideration for cardiac sarcoidosis screening in patients with a history of extra-cardiac manifestations.

Surgical Management of Solitary Extrapulmonary Lymphangioleiomyomatosis in the Mesentery: A Case Report.

Pulmonary lymphangioleiomyomatosis (LAM) is a rare condition characterised by infiltration of the lungs with abnormal smooth muscles and cystic lesions. A rarer form of the condition is extrapulmonary LAM (E-LAM) where the same cystic lesions are found in various organs throughout the body resulting in a variety of symptoms. Given the rarity of E-LAM and the difficulty in diagnosing it, there is little evidence to guide its management both surgically and medically. We describe a case of a 22-year-old female with a nine-month history of abdominal pain found to have a large mesenteric mass during laparoscopy for suspected ovarian torsion. She underwent a laparotomy to exteriorise the mass and dissect it off the mesentery. The mass was removed without compromise to the bowel or mesentery. The patient recovered well without symptoms and was discharged without complications. Histopathology of the mass revealed it to be E-LAM. This case is the first to our knowledge that demonstrates the successful removal of a solitary E-LAM from the mesentery with minimal adverse outcomes and symptomatic relief.

Clinical Outcomes of Transcatheter Aortic Valve Replacement (TAVR) vs Surgical Aortic Valve Replacement (SAVR) in Patients With Sarcoidosis.

Introduction Data regarding clinical outcomes after transcatheter aortic valve replacement (TAVR) vs surgical aortic valve replacement (SAVR) in patients with sarcoidosis is lacking. This study aims to clarify the clinical outcomes of TAVR vs SAVR in patients with sarcoidosis. Methods Data was collected from the National Inpatient Sample database from 2016-2019 using validated ICD-10-CM codes for sarcoidosis, TAVR, and SAVR. Patients were divided into two cohorts: those who underwent TAVR and those who underwent SAVR. Statistical analysis was performed using Pearson's chi-squared test to determine clinical outcomes of TAVR vs SAVR in patients with sarcoidosis. Results The prevalence of sarcoidosis was 0.23% among total study patients (n=142,420,378). After exclusions, the prevalence of TAVR was 650 (49%) and SAVR was 675 (51%) in patients with sarcoidosis. Patients who underwent TAVR were on average older (74 vs 65 years old, p=0.001), and more likely to be female (57 vs 40%, p<0.001) compared to patients who underwent SAVR. The TAVR cohort had higher rates of congestive heart failure (CHF) (77.7 vs 42.2%, p=0.001), chronic kidney disease (CKD) (42.3 vs 24.4% p=0.001), anemia (5.4 vs 2.2%, p=0.004), percutaneous coronary intervention (PCI) (1.5 vs 0%, p=0.004), and hypothyroidism (31.5 vs 16.3%, p=0.001) compared to the SAVR cohort. Inpatient mortality post-procedure was higher in the SAVR cohort compared to the TAVR cohort (15 vs 0, p=0.001). Regarding post-procedure complications, respiratory complications were more common in the SAVR cohort (4.4 vs 0%, p=0.001), while TAVR was associated with a higher incidence of permanent pacemaker (PPM) insertion (2.15 vs 0.8%, p=0.001). There was no statistical difference in the development of acute kidney injury (AKI) (0.8 vs 1.5%, p=0.33), AKI requiring hemodialysis (0 vs. 0.7%, p=0.08), or stroke (0.8 vs 0.7, p=1) post-procedure between the two cohorts. Conclusion This study found that in the sarcoidosis population, TAVR was associated with reduced mortality, shorter hospital length of stay, and lower hospitalization costs in comparison to SAVR.

Systemic lupus erythematosus in pediatric patients: Pulmonary manifestations.

The pulmonary manifestations of Systemic Lupus Erythematosus (SLE) in pediatric patients are poorly understood and the pulmonary manifestations reported from the adult population are generally extrapolated to the pediatric population. In the present work, the review of 228 files was carried out, in which the pulmonary manifestations, symptoms and antibody levels of the patients treated at the Hospital Regional de Alta Especialidad de Ixtapaluca (HRAEI), State of Mexico, Mexico, were identified. Statistical significance between groups was estimated using the Chi-square and Mann-Whitney U test. The main pulmonary manifestations identified were pleurisy (14 %), pulmonary hemorrhage (3.9 %), pulmonary thromboembolism (0.9 %), acute lupus pneumonitis (0.4 %), pulmonary arterial hypertension (0.4 %), and small lung syndrome (0.4 %). While the initial symptomatology was dyspnea with an incidence of 9.6 %, the mean oxygen saturation in the population was 96.87 %. Pleural effusion was identified as the most frequent pulmonary manifestation in radiographic changes. No statistically significant difference was found in antibody levels when comparing the groups. The most common pulmonary manifestation associated with SLE is pleurisy, however, the range of pulmonary manifestations in this type of patient can be very varied, as well as the presentation of each of them.

Radiological and Functional Pulmonary Evolution in Post-COVID-19 Patients: An Observational Study.

COVID-19 has generated a scenario for global health with multiple systemic impairments. This retrospective study evaluated the clinical, radiological, and pulmonary functional evolution in 302 post-COVID-19 patients. Regarding post-COVID-19 pulmonary symptoms, dry cough, dyspnea, and chest pain were the most frequent. Of the associated comorbidities, asthma was more frequent (23.5%). Chest tomography (CT) initially showed a mean pulmonary involvement of 69.7%, and evaluation in the subsequent months showed improvement in the evolutionary image. With less than six months post-pathology, there was a commitment of 37.7% from six to twelve months it was 20%, and after 12 months it was 9.9%. As for most of the sample, 50.3% of the patients presented CT normalization less than six months after infection, 23% were normalized between six and twelve months, and 5.2% presented with normalized images after twelve months, with one remaining. A percentage of 17.3% maintained post-COVID-19 pulmonary residual sequelae. Regarding spirometry, less than six months after pathology, 59.3% of the patients presented regular exam results, 12.3% had their function normalized within six to twelve months, and 6.3% had normal exam results twelve months after their post-pathology evaluation. Only 3.6% of the patients still showed some alteration during this period.

Prevalence of and Factors Associated with Respiratory Symptoms Among Patients with Inflammatory Bowel Disease: A Prospective Study.

BACKGROUND: No large, prospective study has investigated respiratory symptoms in patients with inflammatory bowel diseases. We aimed to describe the prevalence of and factors associated with respiratory symptoms in patients with inflammatory bowel disease. METHODS: In an observational, prospective, cross-sectional study, we evaluated the frequency of respiratory symptoms using a validated self-reporting questionnaire from February 2019 to February 2021 during routine follow-up outpatient visits of patients with inflammatory bowel disease followed in the Gastroenterology Department of the Nancy University Hospital. In case of a positive questionnaire, patients were systematically offered a consultation with a pulmonologist in order to investigate a potential underlying respiratory disease. RESULTS: There were 325 patients included, and 180 patients had a positive questionnaire (144 with Crohn's disease). Of the included patients, 165 (50.8%) presented with respiratory symptoms, with dyspnea being the most frequent symptom (102 patients). There were 102 patients (56.7%) who benefited from a consultation in the pulmonology department: 43 (42.2%) were diagnosed with a respiratory disease, mainly asthma (n = 13) or chronic obstructive pulmonary disease (n = 10). Fourteen patients (13.7%) had obstructive sleep apnea. A body mass index increase, being a smoker or ex-smoker, and having articular extra-intestinal manifestations were independently associated with a higher prevalence of respiratory symptoms. CONCLUSIONS: Half of patients with inflammatory bowel disease reported respiratory symptoms in our study. Patients with inflammatory bowel disease should be systematically screened, as pulmonary disease is frequently present in this population, with specific attention being given to smokers or ex-smokers and patients with extra-articular intestinal manifestations.

We conducted a large-scale, prospective study, finding a high prevalence of respiratory symptoms in patients with inflammatory bowel disease, which led to a variety of respiratory diseases, including asthma and obstructive pulmonary disease. Patients should therefore be systematically screened for pulmonary manifestations.

Extrapulmonary Sarcoidosis With Multi-Organ Involvement Presenting Primarily as Hypercalcemia.

Sarcoidosis is a highly variable disease. The majority of cases affect the lungs, but they can involve other organs as well. Extrapulmonary sarcoidosis is rare, and it can present in many ways. Therefore, the diagnosis can be challenging. We hereby present a case of a patient presenting with hypercalcemia and diagnosed with extrapulmonary sarcoidosis with multi-organ involvement. This article was previously presented as a meeting abstract at the MI-ACP 2022 Annual Scientific Meeting on October 14, 2022.

A Rare Case of Extra-Pulmonary Sarcoidosis With Only Initial Presentation of Hypercalcemia.

Sarcoidosis is a systemic disorder characterized by the aberrant development of granulomatous inflammation within various organs in the body. In over 90% of cases, sarcoidosis typically manifests initially in the intra-thoracic region, characterized by pulmonary involvement or mediastinal lymphadenopathy. It is rare for sarcoidosis to manifest exclusively as extra-thoracic involvement and even more rarely for hypercalcemia to be the only initial sign. We present a case of a 70-year-old female with hypercalcemia and elevated 1,25-dihydroxyvitamin D3 (1,25(OH)2 D) which raised the suspicion of a granulomatous disease. Granulomatous diseases increase levels of 1,25(OH)2 D via the abnormal expression of 1 alpha-hydroxylase enzyme; therefore, these conditions should be considered in the differential diagnosis when encountered with hypercalcemia. PET-CT showed increased FDG uptake in the reticuloendothelial system. An easily accessible inguinal lymph node biopsy was performed which revealed non-necrotizing granulomatous inflammation. Other causes of non-necrotizing granulomatous diseases, cancer, and lymphoma were ruled out, leading to sarcoidosis being considered as a possible diagnosis. When diagnosing sarcoidosis, other potential causes of granulomatous inflammation need to be ruled out definitively via laboratory findings, imaging, and tissue histopathology before initiation of treatment with steroids. Treatment with glucocorticoids remains the mainstay therapy of 1,25(OH)2 D-mediated hypercalcemia associated with sarcoidosis. The patient was accordingly treated with prednisone which led to the normalization of calcium and 1,25(OH)2 D levels within three weeks. Here, we discuss the clinical features and investigations of extra-pulmonary sarcoidosis for early diagnosis and management.

Babesiosis: An Atypical Cause of Respiratory Failure.

Babesiosis is a parasitic infection of the Babesia protozoa, which has been increasing in incidence in endemic areas of the United States. Symptoms of babesiosis can occur on a wide spectrum, from a mild flu-like illness to a fulminant disease course. Known complications of severe cases include intravascular hemolytic anemia and may involve the coagulation system, heart, spleen, kidneys, and in some cases, the lungs. This case report describes an 81-year-old, asplenic female in northern Wisconsin who presented to a hospital with shortness of breath and a non-productive cough. Definitive diagnosis of babesiosis, which was made through both a nucleic acid panel and blood smear, was initially delayed given the rare pulmonary manifestation of babesiosis. When the lungs are involved in the disease course, non-cardiogenic pulmonary edema leading to acute respiratory distress syndrome is among the most commonly seen complications. The pathophysiology of pulmonary involvement has not been made entirely clear but is most likely multifactorial, including the sequelae of changes to both the patient's red blood cells and pulmonary vasculature. This report highlights that atypical tick-borne illnesses like babesiosis should be considered as a cause of acute respiratory failure, particularly in the setting of sepsis and fever. The threshold for parasitic testing should be low in patients in endemic regions with risk factors, including increased age and history of asplenia, as babesiosis frequently has no localizing symptoms to suggest a protozoan infection. As babesiosis incidence continues to rise, prompt diagnosis and proper treatment can prevent severe complications and death in patients.

Lung Imaging in COPD Part 1: Clinical Usefulness.

COPD is a condition characterized by chronic airflow obstruction resulting from chronic bronchitis, emphysema, or both. The clinical picture is usually progressive with respiratory symptoms such as exertional dyspnea and chronic cough. For many years, spirometry was used to establish a diagnosis of COPD. Recent advancements in imaging techniques allow quantitative and qualitative analysis of the lung parenchyma as well as related airways and vascular and extrapulmonary manifestations of COPD. These imaging methods may allow prognostication of disease and shed light on the efficacy of pharmacologic and nonpharmacologic interventions. This is the first of a two-part series of articles on the usefulness of imaging methods in COPD, and it highlights useful information that clinicians can obtain from these imaging studies to make more accurate diagnosis and therapeutic decisions.

An Atypical Case of Extrapulmonary Sarcoidosis.

Sarcoidosis is an idiopathic, inflammatory condition that affects nearly all organs in the body. Lungs are the most frequent and among the earliest sites for detecting it. The most common extrapulmonary manifestations involve the ophthalmic, cardiac, nervous, reticuloendothelial, cutaneous, hepatosplenic, and renal systems. These extrapulmonary manifestations of sarcoid may be misdiagnosed in the absence of the classical pulmonary features, given the high overlap of features with other chronic immunologic diseases. The diagnostic workup to differentiate sarcoid from other similar conditions is extensive, amongst which histology remains a gold standard tool for the diagnosis. Our patient presented with a chronic history of multiple vague complaints including nausea, vomiting, progressive malaise, vision changes, and weight loss. After extensive workup, a diagnosis of sarcoidosis along with multiple rare extrapulmonary involvements was made. The authors highlight essential implications including primary practice goals to avoid misdiagnosis or missed sarcoid diagnoses thus helping improve clinical outcomes in similar populations.

Pulmonary Manifestations of Inflammatory Bowel Disease.

The frequency of extraintestinal manifestations of inflammatory bowel disease (IBD) is observed in most of the patients. Extraintestinal manifestations of IBD have been implicated in all the anatomic sites of the pulmonary tree, and include airway inflammation of the bronchi and bronchioles and parenchymal inflammation. There is a wide range of pulmonary manifestations in IBD, and most of them look a lot like other diseases. Doctors should keep a track of these conditions to avoid unnecessary complications and to provide an early diagnosis with correct and efficient treatment. IBD-related respiratory disorders are treated depending on the patient, but in most of them, steroids are generally chosen first. Steroids, both inhalational and systemic, are the primary approach. Antibiotics could also be given if the patient has infections or suppuration, usually followed by surgeries. However, drug-induced complications and toxicity should be kept in mind.

The Long-Term Use of Methotrexate Monotherapy as a Steroid-Sparing Agent for Patients With Pulmonary Sarcoidosis.

This report aims to determine the benefits of long-term use of methotrexate as a steroid-sparing agent for a patient with pulmonary sarcoidosis who cannot tolerate the glycemic effects of steroids. A search for articles using the medical subject heading terms "methotrexate" and "sarcoidosis" on PubMed involving clinical trials evaluating the therapeutic effect and toxic profile of methotrexate as a steroid-sparing agent in treating pulmonary and/or extrapulmonary symptoms of sarcoidosis was included. The literature review indicates that methotrexate is an alternative treatment for sarcoidosis, allowing the patient to avoid the long-term side effects of steroids while achieving similar rates of treatment and remission. Patients in several research studies were able to taper their steroid dosage over time when methotrexate was concurrently prescribed for the treatment of pulmonary sarcoidosis. The critical appraisal of one of the studies reviewed indicates methotrexate as a single agent in treating patients with chronic progressive pulmonary sarcoidosis, serving as a steroid alternative. Methotrexate was safe and effective for a duration of 6-24 months, with patients experiencing definite improvements in pulmonary function tests. However, not all patients in this open prospective, real-life, single-center trial demonstrated improvement. Thus, a review of the current literature is often necessary to help guide clinical decision-making for patients with chronic diseases like sarcoidosis and to determine patient characteristics of methotrexate responders.