Idiopathic cough

BACKGROUND: Bronchiectasis is a chronic airway disease characterized by permanent and irreversible abnormal dilatation of bronchi. Several studies have reported the development of bronchiectasis after renal transplantation (RT), but no prospective study specifically assessed bronchiectasis in this population. This study aimed to compare features of patients with bronchiectasis associated with RT to those with idiopathic bronchiectasis. METHODS: Nineteen patients with bronchiectasis associated with RT (RT-B group) and 23 patients with idiopathic bronchiectasis (IB group) were prospectively included in this monocentric cross-sectional study...

Idiopathic pulmonary fibrosis (IPF) is a continuous, progressive, and lethal age-related respiratory disease. It is characterized by condensed and rigid lung tissue, which leads to a decline in the normal functioning of the lungs. The pathophysiology of IPF has still not been completely elucidated, so current strategies are lagging behind with respect to improving the condition of patients with IPF and increasing their survival rate. The desire for a better understanding of the pathobiology of IPF and its early detection has led to the identification of various biomarkers associated with IPF...

Stevens-Johnson syndrome and toxic epidermal necrolysis are potentially life-threatening cutaneous diseases that are rarely seen in the pediatric population. We describe a case of a 7-year-old female who presented with fever, cough, mucosal erosions, and a widespread maculopapular eruption with flaccid bullae for 4 days. She had no sick contacts and no new medications, supplements, or vitamins. She then rapidly developed ulcerations in her oral mucosa and genital area, numerous scattered tense bullae throughout her body, and patchy areas of desquamation...

Pulmonary arterial hypertension (PAH) is a perilous disease that precipitates right ventricular hypertrophy, induces right heart failure, and exerts deleterious ramifications on prognostic outcomes. The establishment of atrial communication can create a right-to-left shunt, thereby ameliorating hemodynamic parameters. Previous reports suggested that opening of a patent foramen ovale (PFO) was common in patients with severe PAH, but exhibited no discernible impact on long-term survival. We reported the case of a 39-year-old man with severe idiopathic PAH, who underwent reopening of the PFO due to severe cough and hemoptysis, followed by a marked amelioration in symptoms and a substantial decrease in pulmonary arterial pressure...

Cerebellar ataxia, neuropathy, and vestibular areflexia syndrome (CANVAS) is an autosomal recessive multisystem neurologic disorder caused by biallelic intronic repeats in RFC1. Although the phenotype of CANVAS has been expanding via diagnostic case accumulation, there are scant pedigree analyses to reveal disease penetrance, intergenerational fluctuations in repeat length, or clinical phenomena (including heterozygous carriers). We identified biallelic RFC1 ACAGG expansions of 1000 ~ repeats in three affected siblings having sensorimotor neuronopathy with spinocerebellar atrophy initially presenting with painful muscle cramps and paroxysmal dry cough...

In this issue of NEJM Evidence , Maher et al.1 report the results of a randomized, controlled, 22-day treatment crossover trial comparing the antitussive effect of extended-release nalbuphine, an opioid agonist-antagonist, with placebo in a cohort of patients with definite or probable idiopathic pulmonary fibrosis (IPF). In this small, short-term trial of 38 evaluable patients, the active drug was associated with a 75.1% reduction in daytime objective cough frequency (the primary outcome) compared with a 22...

Nalbuphine for Cough with Idiopathic Pulmonary FibrosisIn patients with idiopathic pulmonary fibrosis, cough may have a negative impact on daily life. In a randomized, 22-day treatment period, placebo-controlled, crossover trial, extended-release nalbuphine (NAL ER), an opioid agonist-antagonist, was compared to placebo for cough control and adverse effects. During active treatment there was a 75.1% reduction in daytime objective cough frequency compared with 22.6% in the placebo treatment period. Nausea, fatigue, constipation, and dizziness were more common with NAL ER than with placebo...

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Vanishing lung syndrome (VLS) also known as type I bullae disease or idiopathic bullous disease is characterized by giant emphysematous bullae that commonly develop in the upper lobes, occupying at least one-third of a hemithorax. It is a progressive and irreversible condition that involves pulmonary parenchymal destruction and alveolar dilation. It is commonly associated with middle-aged tobacco smokers, habitual marijuana users, and those with alpha-1-antitrypsin deficiency. This case involves an incarcerated male in his 30s with chronic marijuana smoking who presented with a three-month history of right-sided chest pain accompanied by cough, hemoptysis, fever, and weight loss...

INTRODUCTION: All that wheezes is not asthma. Although, asthma is the most common cause of wheeze and cough in children and adults, it is often attributed inappropriately to these symptoms from other causes. AIM: We illustrate through this manuscript three rare causes of wheeze in adults, misdiagnosis as asthma. OBSERVATIONS: The three reported cases were misdiagnosed as difficult-to-treat asthma. They were found as mimicking asthma. Final diagnoses were localized tracheobronchial amyloidosis, aortic arch anomalies, and idiopathic chronic eosinophilic pneumonia...

BACKGROUND: Idiopathic pulmonary fibrosis is a progressive fibrotic lung disease, with most patients reporting cough. Currently, there are no proven treatments. We examined the use of low dose controlled-release morphine compared with placebo as an antitussive therapy in individuals with idiopathic pulmonary fibrosis. METHODS: The PACIFY COUGH study is a phase 2, multicentre, randomised, double-blind, placebo-controlled, two-way crossover trial done in three specialist centres in the UK...

Palliative care has emerged as a crucial aspect of comprehensive healthcare, particularly in respiratory medicine. This review navigates the intricate landscape of palliative care in the context of respiratory diseases, including chronic obstructive pulmonary disease (COPD), idiopathic pulmonary fibrosis (IPF), and lung cancer. The exploration begins with a comprehensive examination of palliative care's definition, significance, and purpose in respiratory medicine. It progresses to understanding common respiratory diseases, their impact on patients' quality of life, and the nuances of disease progression and prognosis...

Invasive pulmonary aspergillosis (IPA) and invasive aspergillosis (IA) are two examples of the broad clinical spectrum of Aspergillus infection. It mainly affects severely immunocompromised hosts, while immunocompetent people can sometimes be affected, especially those receiving treatment in the intensive care unit (ICU) for emergency cases with few instances of chronic cases. The risk factors in ICU patients for aspergillosis include intubated patients receiving hot and humidified air, viral infections like covid, and influenza, and diseases like diabetes, chronic obstructive pulmonary disease, etc...

We diagnosed and treated a case of Primary SjoGren's Syndrome with Idiopathic Pulmonary Fibrosis (IPF) in a 65 years old woman who presented with dyspnoea and multiple joint pains for 5 years and remained undiagnosed. She had variable presentation and was initially established as a case of mixed connective tissue disease which consists of Systemic Lupus Erythematosus (SLE), Systemic Sclerosis and Dermatomyositis. She complained of xerostomia, xerophthalmia, difficulty in opening mouth, progressive dysphagia with solid foods and raynaud's phenomenon...

INTRODUCTION: Many patients with interstitial lung diseases (ILDs), especially fibrotic ILDs, experience chronic cough. It negatively impacts both physical and psychological well-being. Effective treatment options are limited. AREAS COVERED: The pathophysiology of chronic cough in IPF is complex and involves multiple mechanisms, including mechanical distortion of airways, parenchyma, and nerve fibers. The pathophysiology of cough in other fibrosing ILDs is poorly understood and involves various pathways...

We present the case of a 40-year-old female who underwent several insertions of ventriculoperitoneal (VP) shunts as a part of the treatment for idiopathic intracranial hypertension (IIH). Several years after the insertion of the last VP shunt, the patient started experiencing shortness of breath (SOB) and cough; after further assessment, it was noted on computed tomography (CT) scan that the VP shunt had migrated into the right lower lobe of the lung and perforated the distal left main bronchus. The shunt was successfully retrieved using bronchoscopy under general anesthesia, after which the patient had a complete resolution of symptoms...

BACKGROUND: Dyspnoea and cough can have a profound impact on the lives of patients with pulmonary fibrosis. We investigated the effects of nintedanib on the symptoms and impact of pulmonary fibrosis in patients with progressive pulmonary fibrosis (PPF) in the INBUILD trial using the Living with Pulmonary Fibrosis (L-PF) questionnaire. METHODS: Patients had a fibrosing interstitial lung disease (ILD) (other than idiopathic pulmonary fibrosis) of >10% extent on high-resolution computed tomography (HRCT) and met criteria for ILD progression within the prior 24 months...

In recent years, there has been a substantial increase in the development of antitussive therapies and the first new therapy, gefapixant has been licenced in Europe. This review describes current unlicenced treatments for chronic cough and details treatments currently in development for refractory chronic cough and cough in idiopathic pulmonary fibrosis, as well as compounds previously explored.

A 73-year-old Japanese woman with productive cough and dyspnoea on exertion was admitted, and she showed neutropenia and pulmonary reticular opacities and axial and mediastinal lymphadenopathies on chest computed tomography. The clinical findings and surgical lung and lymph node biopsies were diagnostic for idiopathic multicentric Castleman's disease (iMCD) complicated by secondary autoimmune neutropenia (AIN). iMCD is often complicated with hematologic disorders, however, iMCD complicated with AIN has not been reported; therefore, if iMCD is accompanied by neutropenia, the anti-neutrophil antibodies should be measured...

Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibro-inflammatory disease with the potential to involve virtually all organs, including the pancreas, kidneys, lungs, and pleura, amongst others. IgG4-RD pleural involvement may cause diverse complications such as pleural effusion, pleural thickening, pleural nodules, and additional lesions, which can be presented in many clinical diseases. However, isolated cases of pleurisy are still rare in IgG4-RD. We report a 72-year-old patient who was admitted to our hospital with cough, expectoration, and fatigue...