Lipoid pneumonia induced by aspiration of liquid paraffin.

INTRODUCTION: This case report describes a case of exogenous lipoid pneumonia (ELP) resulting from the inhalation of a lipoid substance. Lipoid pneumonia, also known as cholesterol pneumonia or golden pneumonia, is an uncommon inflammatory lung disease characterized by the presence of lipid-laden macrophages in the alveolar walls and lung interstitial tissue. Exogenous lipoid pneumonia occurs when substances containing lipids enter the airways through aspiration or inhalation, triggering an inflammatory response. CASE REPORT: The patient in this case study was an 83-year-old woman with hypertension and diabetes mellitus who had been using paraffin oil as a mouthwash for an extended period. The diagnosis of exogenous lipoid pneumonia was established based on the patient's history of exposure to liquid paraffin oil, typical radiological findings, and histopathological examination.

Concomitant Nitrofurantoin-Induced Autoimmune Hepatitis and Interstitial Lung Disease.

Antibiotics are known to cause adverse reactions, but multiple organ involvement associated with nonspecific symptoms can lead to a delay in diagnosis. A definitive correlation between each toxin and its effects is difficult to establish due to concomitant potential toxins in the circulation. This article highlights an uncommon case of concomitant nitrofurantoin-induced autoimmune hepatitis and lung fibrosis that fulfills the definitive clinical criteria for diagnosis, presenting histological, imagiological, and immunological evidence of nitrofurantoin-induced toxicity. It occurred in a 68-year-old woman with extended nitrofurantoin intake for urinary tract infection prophylaxis who presented with progressive exercise dyspnea and jaundice. Similar published cases are also reviewed in this article.

Os antibióticos são causas conhecidas de reações adversas, mas o envolvimento multiorgânico associado à sintomatologia inespecífica pode conduzir ao atraso diagnóstico. Devido às potenciais toxinas concomitantemente em circulação, é muitas vezes difícil estabelecer uma correlação definitiva entre cada toxina e os seus efeitos.Este artigo salienta um caso incomum de hepatite autoimune e fibrose pulmonar induzidas pela nitrofurantoína e que cumpre critérios definitivos de diagnóstico, apresentando-se dados histológicos, imagiológicos e imunológicos da toxicidade induzida pela nitrofurantoína.O caso ocorre numa mulher de 68 anos de idade, com toma prolongada de nitrofurantoína como profilaxia de infeção urinária, e que se apresenta com dispneia de esforço progressiva e icterícia. O artigo faz ainda uma revisão de casos semelhantes publicados.

Niemann-Pick Type B: A Rare Cause of Interstitial Lung Disease.

Niemann-Pick disease (NPD) is a rare lysosomal storage disease (LSD) with multisystemic involvement. The disease is heterogeneous and classified into three subtypes: type A and B result from deficient acid sphingomyelinase activity and leads to the accumulation of sphingomyelin and type C is a genetically different disease resulting from defective intracellular trafficking of cholesterol with accumulation of glycosphingolipids. Type A is generally a neurodegenerative disease and is fatal in infancy. Type B is a less severe form characterized by pulmonary involvement, hepatosplenomegaly, hyperlipidemia and most patients live into adulthood. In type C, clinical presentation is dominated with neurological involvement. Pulmonary involvement occurs in all three types of Niemann-Pick but most frequently in type B. Clinical manifestations range from a lack of symptoms to respiratory failure, and respiratory symptoms are usually mild with recurrent cough, dyspnoea on exertion and recurrent respiratory infections. Interstitial lung disease (ILD) is the most prominent feature with slow progression, characterized by worsening pulmonary function tests. In recent years, enzyme replacement therapy has shown promising results in clinical trials, such as improvement in organomegaly and pulmonary involvement with the potential to improve patients' lives. We present three cases of NPD with pulmonary involvement, each exhibiting a different pattern of ILD and evaluate therapeutic options.

Pulmonary MALT lymphoma associated with interstitial pulmonary disease.

Interstitial lung disease (ILD) and low-grade Mucosa-associated B-cell lymphoma (MALT lymphoma) are two different disorders of the respiratory system. In some cases, pulmonary MALT lymphoma is seen presenting with interstitial lung disease. We report a case of 42-year-old man presenting with a pulmonary MALT lymphoma associated with interstitial lung disease.

Advance in ABCA3 gene mutation and related pediatric pulmonary diseases / 国际儿科学杂志

ATP-binding cassette transporter A3(ABCA3) gene mutation is one of the important causes of severe respiratory distress syndrome and interstitial lung disease in children and adults.Clinical phenotypes vary dramatically among patients with ABCA3 mutations.So far, the genotype-phenotype correlation is not entirely clear.The association between the possible factors that influence the phenotypes, such as the environment, infection and diseases remains to be studied.There is no specific treatment for the diseases caused by the mutations.Present models for studying ABCA3 mutations in vitro are still to be improved.This article focuses on reviewing the structure, genetics and the research progress of treatment of ABCA3 gene mutation related pediatric diseases, in order to provide experience and ideas for further researches and treatment of the diseases caused by ABCA3 gene mutation.

Neumonía intersticial linfocítica en una paciente con lupus eritematoso sistémico y síndrome de Sjögren secundario: reporte de caso y revisión de la literatura / Lymphocytic interstitial pneumonia in a patient with systemic lupus erythematosus and secondary Sjögren's syndrome: Case report and literature review

RESUMEN La neumonía intersticial linfocítica es una complicación, poco frecuente, asociada con el lupus eritematoso sistémico, sin embargo, con gran impacto en la calidad de vida. Se asocia con la presencia de anti Ro/SSA, anti La/SSB y con el diagnóstico de síndrome de Sjögren secundario. No es clara la estrategia terapéutica y la información existente está basada en reportes de caso sin disponibilidad de estudios adecuadamente diseñados. En el presente documento se expone el caso de una paciente con lupus eritematoso sistémico y síndrome de Sjögren secundario, que presentó una enfermedad pulmonar intersticial con características de neumonía intersticial linfocítica.

ABSTRACT Lymphocytic interstitial pneumonia is an infrequent complication associated with systemic lupus erythematosus and has a great impact on quality of life. It is associated with the presence of anti-Ro/SSA, anti-La/SSB, and the diagnosis of secondary Sjögren Syndrome. Its therapeutic strategy is not clear, and the existing information is based on case reports, with there being no properly designed studies available. The case is presented here of a patient with systemic lupus erythematosus and secondary Sjögren's syndrome, who also presented with interstitial lung disease with characteristics of lymphocytic interstitial pneumonia.

Non-specific impairment of Lung Function on Spirometery in Patients with Chronic Hepatitis-C.

OBJECTIVES: To document frequency of non-specific impairment of lung functions (NILF) in patients of HCV and to compare according to gender, genotype, liver fibrosis score and smoking status. METHODS: Patients of chronic hepatitis C were included after informed consent. Demographic data was recorded, and they underwent baseline investigations, fibroscan, abdominal ultrasound and PFT. Patients were segregated on basis of gender, fibroscan stages and smoking status. NILF was labelled if any two of three criteria are fulfilled (a) FVC < 80% of Predicted, (b) FEV1 < 80% Predicted, (c) FEV1/FVC ≥ 70. RESULTS: Two hundred thirty four patients were of chronic HCV who fulfilled the selection criteria were inducted in study. These included 49.6% males and 50.4% females. There were 15.0% smokers, 16.2% were ex-smokers while 68.8% were never smokers. NILF was present in 130 (55.6%) out of which 61.5% were female and 38.5% were male (p <0.001), its presence in smokers was 56.2% and in never smokers was 55.3% (p=0.507). Presence of NILF increased with Fibroscan stages from F1 to F4 (p <0.001). CONCLUSIONS: NILF pattern on spirometry with normal chest radiograph is common among HCV patients. It was found more common in females and frequency increased progressively with fibro scan stages.

Hepatosplenomegaly, pneumopathy, bone changes and fronto-temporal dementia: Niemann-Pick type B and SQSTM1-associated Paget's disease in the same individual.

Data from exome sequencing show that a proportion of individuals in whom a genetic disorder is suspected turn out to have not one, but two to four distinct ones. This may require an evolution in our diagnostic attitude towards individuals with complex disorders. We report a patient with splenomegaly, pneumopathy, bone changes and fronto-temporal dementia (FTD). "Sea-blue histiocytes" in his bone marrow pointed to a lysosomal storage disease. Homozygosity for a pathogenic mutation in the SMPD1 gene confirmed Niemann-Pick disease type B (NPD-B). Mild cognitive impairment and abnormal brain FDG PET were consistent with FTD. We initially tried to fit the skeletal and neurologic phenotype into the NPD-B diagnosis. However, additional studies revealed a pathogenic mutation in the SQSTM1 gene. Thus, our patient had two distinct diseases; NPD-B, and Paget's disease of bone with FTD. The subsequent finding of a mutation in SQSTM1 gene ended our struggle to explain the combination of findings by a singular "unifying" diagnosis and allowed us to make specific therapeutic decisions. SQSTM1 mutations have been reported in association with FTD, possibly because of defective autophagy. Bisphosphonates may be beneficial for PDB, but since they are known to inhibit acid sphingomyelinase activity, we refrained from using them in this patient. While the principle of looking for unifying diagnosis remains valid, physicians should consider the possibility of co-existing multiple diagnoses when clinical features are difficult to explain by a single one. Accurate diagnostic work-up can guide genetic counseling but also lead to better medical management.

A 64-year-old woman with interstitial lung disease and positive antibodies against aminoacyl-transfer RNA synthetases in the absence of myositis: presentation of an anti-PL-12 positive antisynthetase syndrome.

Introduction The antisynthetase syndrome is a rare autoimmune disease described by the presence of inflammatory myositis, interstitial lung disease and antibodies against aminoacyl-transfer RNA synthetases. Interstitial lung disease can be the only manifestation in the absence of an inflammatory myositis. Other clinical signs are Raynaud phenomenon, hyperkeratotic skin lesions, fever and inflammatory polyarthritis. Case presentation We report the case of a 64-year old woman who complained of a dry cough, progressive dyspnea and arthralgia since 2 years, with no other systemic symptoms. High resolution computed tomography (HRCT) of the thorax showed the presence of bilateral ground glass opacities, reticular opacities and some traction bronchiectasis. Further biochemical testing revealed the presence of anti-PL12 antibodies. Management The diagnosis of antisynthetase syndrome was made and the patient was treated with steroids and azathioprine with a good response. Conclusion The search for antisynthetase antibodies should always be considered in patients with an interstitial lung disease without any other clinical symptoms or signs of an underlying connective tissue disease.

Neumonitis intersticial descamativa en el paciente pediátrico / Desquamative interstitial penumonitis in a pediatric patient

Introducción: la neumonitis intersticial descamativa es una entidad caracterizada en la clínica por mostrar tos, disnea, cianosis e hipercapnia, con un patrón restrictivo de las pruebas funcionales respiratorias, debido a la presencia de fibrosis pulmonar, cuya frecuencia es inusual en Pediatría. Presentación del caso: adolescente que fue remitida al Hospital Docente Pediátrico del Cerro por sospecha de dengue, al referir fiebre de 38 °C de 3 días de evolución, acompañada de dolores musculares en miembros inferiores, escalofríos y cefalea, por lo cual fue internada en la sala de misceláneas. Durante su evolución mostró dificultad respiratoria, tos seca, taquipnea, taquicardia y disminución del murmullo vesicular en la base del pulmón izquierdo. Se observó en la radiografía de tórax una opacidad en dicha zona y fue tratada con antibióticos. En etapa posterior se trasladó a la Unidad de Cuidados Intensivos por ocurrir un incremento de las lesiones pulmonares e insuficiencia respiratoria; por ello, se indicó ventilación mecánica, variedad presión controlada. Posteriormente se aisló en hemocultivo y secreciones bronquiales, Pseudomona Stutzeri, evento considerado como una sepsis asociada a cuidados sanitarios. Se planteó un distrés respiratorio del adulto en niños que no involucionó, y falleció en un cuadro de insuficiencia respiratoria a los 19 días de estadía. Conclusiones: esta paciente mostró síntomas y signos sugestivos de una infección pulmonar bacteriana de evolución tórpida. Los hallazgos necrópsicos describen la presencia de una bronconeumonía bacteriana como causa directa, y una neumonitis intersticial descamativa, como entidad básica del fallecimiento(AU)

Introduction: desquamative interstitial pneumonitis is a characterized condition in the clinical field since it shows cough, dysnea, cyanosis and hypercapnia, with a restrictive pattern of the functional respiratory tests due to the presence of pulmonary fibrosis that is unusual in pediatrics. Case presentation: a female adolescent was referred to the pediatric teaching hospital of Cerro on suspicion of dengue since she presented with 38 °C for three days, accompanied with muscle aches in lower limbs, chills and headache. She was admitted to a general ward. During her progression, she showed respiratory distress, unproductive cough, tachypnea, tachycardia and reduction of vesicular murmur in the left lung basis. The thoracic X ray showed opacity in the area and was treated with antibiotics. In a later phase, she was moced to the intensive care unit due to increase in pulmonary lesions and respiratory failure. She was also under mechanical ventilation with controlled pressure. Later, Pseudomona Stutzeri was isolated in blood culture and bronchial secretions, an event considered to be health care-associated sepsis. It was stated that this case was a respiratory distress of adult in a child that evolved and finally the adolescent died of respiratory failure 19 days after her hospitalization. Conclusions: this patient showed symptoms and signs suggestive of bacterial pulmonary infection of torpid progression. The necropsis finding describe the presence of bacterial bronchopneumonia as a direct cause and desquamative interstitial pneumonitis as the basic condition for death(AU)

Neumonitis intersticial descamativa en el paciente pediátrico / Desquamative interstitial penumonitis in a pediatric patient

Introducción: la neumonitis intersticial descamativa es una entidad caracterizada en la clínica por mostrar tos, disnea, cianosis e hipercapnia, con un patrón restrictivo de las pruebas funcionales respiratorias, debido a la presencia de fibrosis pulmonar, cuya frecuencia es inusual en Pediatría.Presentación del caso: adolescente que fue remitida al Hospital Docente Pediátrico del Cerro por sospecha de dengue, al referir fiebre de 38 °C de 3 días de evolución, acompañada de dolores musculares en miembros inferiores, escalofríos y cefalea, por lo cual fue internada en la sala de misceláneas. Durante su evolución mostró dificultad respiratoria, tos seca, taquipnea, taquicardia y disminución del murmullo vesicular en la base del pulmón izquierdo. Se observó en la radiografía de tórax una opacidad en dicha zona y fue tratada con antibióticos. En etapa posterior se trasladó a la Unidad de Cuidados Intensivos por ocurrir un incremento de las lesiones pulmonares e insuficiencia respiratoria; por ello, se indicó ventilación mecánica, variedad presión controlada. Posteriormente se aisló en hemocultivo y secreciones bronquiales, Pseudomona Stutzeri, evento considerado como una sepsis asociada a cuidados sanitarios. Se planteó un distrés respiratorio del adulto en niños que no involucionó, y falleció en un cuadro de insuficiencia respiratoria a los 19 días de estadía.Conclusiones: esta paciente mostró síntomas y signos sugestivos de una infección pulmonar bacteriana de evolución tórpida. Los hallazgos necrópsicos describen la presencia de una bronconeumonía bacteriana como causa directa, y una neumonitis intersticial descamativa, como entidad básica del fallecimiento(AU)

[MRI of the pulmonary parenchyma: Towards clinical applicability?] / IRM du parenchyme pulmonaire : vers une application clinique ?

Lung parenchyma has long been considered out of the scope of magnetic resonance imaging (MRI) clinical applicability. However, technological advances have emerged to soluce the technical difficulties and thus, applications in clinical practice have become realistic. Nevertheless, various approaches have been proposed and there is a need to synthetize the most recent literature data in order to envision a rationale to build lung MR protocols for clinical use. In addition, these technological innovations may modify the usual paradigms of lung MRI, which are still not consensual. Thus, lung MR protocols appear to be heterogeneous across expert centers in the current context. In this literature review, we ought to describe a rationale on the need to get an alternative to ionizing imaging modalities, in particular in the follow-up of patients with chronic lung diseases. We will describe the most recent technical advances regarding both morphological and functional MRI. Finally, we will conclude on the clinical applicability of MRI of the pulmonary parenchyma, as a routine or research tool.

Pulmonary Hypertension Due to Common Respiratory Conditions: Classification, Evaluation and Management Strategies.

Pulmonary hypertension (PH) due to chronic respiratory disease and/or hypoxia is classified as World Health Organization (WHO) Group III pulmonary hypertension. The patients most commonly encountered in clinical practice with group III PH include those with chronic obstructive lung disease (COPD), diffuse parenchymal lung disease, and sleep-disordered breathing. The purpose of this review is to outline the variable clinical significance of pulmonary hypertension in the most common pulmonary disease states and how a clinician may approach the management of these patients.

Is there still hope for single therapies: how do we set up experimental systems to efficiently test combination therapies?

Severe asthma, chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis (IPF) are chronic lung diseases with a clear need for development of new and more efficient therapy. In preclinical research, the mouse model has been instrumental in advancing our knowledge of the biology and immunology. However, it has been proven rather difficult and time consuming to develop new treatments that can impact on the clinical course of these diseases. Many challenges need to be overcome for upgrading the quality of currently available experimental disease models in order to enhance the translation rate of basic research to clinical practice. Establishment of transgenic mouse overexpressing disease-causing genes may provide tools to discover new pathological pathways and to evaluate the possibility of molecular targeted therapy in chronic lung diseases. Personalized medicine, if developed, might be the solution for 'disease heterogeneity' and for improving clinical outcome.

Enfermedad pulmonar intersticial como manifestación inicial de síndrome antisintetasa / Interstitial lung disease as initial manifestation of anti-synthetase syndrome

Se reporta el caso de un paciente que presentó un síndrome antisintetasa en el cual la miopatía fue precedida por EPID grave de instalación rápida y buena respuesta al manejo inmunosupresor con corticosteroides, ciclofosfamida y azatioprina. El cuadro clínico inicial con fiebre, infiltrados pulmonares, SDRA y ausencia de miopatía fue muy sugestivo de infección.Palabras clave: miopatía inflamatoria, enfermedad pulmonar intersticial, síndrome antisintetasa.

We report a patient with antisintetase síndrome with rapid and progressive interstitial difuse pulmonary disease preceding the muscular weakness. The patient was successfully treated with steroids, cyclophosphamide and azathioprine. The initial compromise: fever, dispnoea and pulmonary infiltrates without miopathy was misdiagnosed as pneumonia.Key words: inflamatory miopathy, interstitial pulmonary disease, antisintetase syndrome.