Interstitial lung involvement in systemic sclerosis / Compromiso intersticial pulmonar en la esclerosis sistémica

ABSTRACT Introduction: Systemic sclerosis can involve the lung parenchyma leading to serious complications and even death. Objectives: To describe the most relevant aspects of interstitial lung disease related to systemic sclerosis emphasizing diagnosis and treatment. Materials and methods: A literature review was performed searching in the databases Medline and EMBASE using the MeSH terms «Scleroderma, Systemic", «Lung Diseases, Interstitial¼ and «Pulmonary Fibrosis¼ Results and conclusions: Interstitial lung disease is a common clinical manifestation of systemic sclerosis and one of the main causes of death. Treatment options are limited and have a modest effect in most of the cases.

RESUMEN Introducción: La esclerosis sistémica puede potencialmente comprometer el parénquima pulmonar, llevando a serias complicaciones e incluso a la muerte. Objetivos: Describir los aspectos más relevantes en cuanto a las generalidades de la enfermedad pulmonar intersticial en esclerosis sistémica, su diagnóstico y su tratamiento. Materiales y métodos: Se realizó una búsqueda de literatura en las bases de datos Medline y EMBASE utilizando los términos MeSH «Scleroderma, Systemic¼, «Lung Diseases, Interstitial¼ y «Pulmonary Fibrosis¼. Resultados y conclusiones: La enfermedad pulmonar intersticial es una manifestación frecuente de la esclerosis sistémica y una de las principales causas de muerte en los pacientes que la padecen. Las opciones terapéuticas son limitadas y su efecto es, en muchos casos, modesto.

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)

Polyarteritis Nodosa Associated with Interstitial Pulmonary Fibrosis / 대한피부과학회지

Classic polyarteritis nodosa (PAN) involves small-to-medium-sized muscular arteries in multiple organ systems. It presents in combination with skin diseases, myalgia, hypertension, abdominal pain, or neuropathy. Although PAN involves the muscles, kidneys, nerves, and gastrointestinal tract, lung involvement presenting as an interstitial pulmonary fibrosis has rarely been reported. A 71-year-old man presented to our clinic complaining of weakness in his lower legs and painful subcutaneous nodules that began to develop a fortnight before he visited the clinic. He had been diagnosed with mononeuritis multiplex, interstitial pulmonary fibrosis, and a cerebral infarction in 2006. All of the preexisting diseases had become aggravated when his lower leg pain began. Laboratory tests for cytoplasmic antineutrophil cytoplasmic antibodies (ANCA), perinuclear-ANCA, and other autoimmune markers were within the normal limits. A biopsy showed mononuclear infiltrations with fibrinoid necrosis involving the medium-sized arteries. Computed tomographic scanning of the chest revealed an increase in parenchymal ground-glass opacity. The histological findings, laboratory tests, and the clinical history were consistent with PAN that was associated with interstitial pulmonary fibrosis. We report this rare case of PAN associated with interstitial pulmonary fibrosis.

Discussion on Clotting Mechanism of Interstitial Pulmonary Fibrosis / 世界科学技术-中医药现代化

Pulmonary fibrosis is a common respiratory disease in the clinic. Until now, the pathogenesis is still un-clear. Using clotting mechanism as the starting point, this article mainly explored abnormal changes of the coagula-tion - fibrinolysis system in the development of pulmonary fibrosis. The effective treatment through the activation of blood circulation to remove stasis in traditional Chinese medicine (TCM) point of view was also observed on the man-agement of interstitial pulmonary fibrosis. It considered that to carry out the anticoagulant therapy for abnormal coag-ulation, which may become a new target for clinical treatment of interstitial lung diseases. It provided new ideas and theoretical support for clinical treatment of pulmonary interstitial fibrosis.

The Role of HRCT in the Follow-Up Evaluation of Diffuse Interstitial Pulmonary Fibrosis / 결핵

BACKGROUND: Because of the power of HRCT to detect the minute changes in lung parenchyme and the advantage of noninvasiveness, it may be the ideal method of follow-up evaluation of the patients with diffuse interstitial pulmonary fibrosis. So the aim of the study is to find out whether the change of HRCT during the course of the disease can represent the change in symptom and pulmonary function rest. METHODS: Thirteen patients with diagnosis of diffuse interstitial pulmonary fibrosis who had more than two times of HRCT during the course of disease were studied. The extent of the disease in HRCT was measured at three levels using image analyzer The ralationship between the percent change in the disease extent in HRCT, symptom score, and pulmonary function testis were analysed. RESULTS: The change of disease extent in HRCT has good correlation with the change in dyspnea and diffusion capacity(r=0.716, p=0.0012). But there was no correlation between the change of HRCT and FVC or TLC. Also significant inverse correlation was noted between the change in dyspnea and diffusion capacity(r=-0.707, p=0.0047). CONCLUSION: These data suggest that HRCT may be a good method of follow-up in diffuse interstitial pulmonary fibrosis.

An Ultrastructural Study of Bleomycin-Induced Interstitial Pulmonary Fibrosis in the Rat

This study was carried out to investigate the mechanisms of interstitial pulmonary fibrosis of rats after the intratracheal administration of bleomycin. Both lungs after bleomycin injection were examined by light and electron microscopy. The results are as follows: Light microscopically, 1 or 2 weeks after bleomycin injection acute and chronic inflammatory infiltrates and edema in the interstitium and alveolar spaces were observed. Proliferation of alveolar type II pneumocytes was also found at 4 to 6 weeks after bleomycin injection, chronic inflammatory infiltrates with interstitial fibrous thickening were noted. Electron microscopically, the number of type II pneumocytes and irregular lamellar bodies were increased and blunted microvilli were noted at 2 weeks. 4 to 8 weeks, proliferation of fibroblasts with deposition of abundant collagen fibrils in the thickened interstitium revealing irregular or collapsed alveolar spaces were observed. Based on these findings, it can be concluded that bleomycin-induced interstitial pulmonary fibrosis is considered to pass from an early acute inflammation of the interstitium and alveolar spaces to an interstitial fibroblast proliferation and collagen deposition to the length of the period after injection.