Interstitial Pneumonia in Patients with Idhiopatic Thrombocytopenic Purpura.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3953-3953
Author(s):  
Vincenzo Fontana ◽  
Elio Donna ◽  
Pamela Dudkiewicz ◽  
Gabriella Lander ◽  
Yeon S. Ahn

Abstract INTRODUCTION: Idhiopatic thrombocytopenic purpura (ITP) is an autoimmune disease characterized by a premature destruction of platelets by macrophage, especially in the spleen. However in some cases, platelet sequestration and destruction may occur in other organs. Chromium labeled platelet sequestration study revealed that liver or precordial area are prominent sites of sequestration in some cases, suggesting that the lung might be the site in certain cases. Some cases of interstitial pneumonia are associated with immunologic injury to the lung and seen in patients with some autoimmune diseases, infections, drugs and transfusion related acute lung injury (TRALI) in which transfusions of platelets and blood products induce acute lung injury due to sequestration of platelets and neutrophils in lungs, sometimes leading to ARDS. We describe here an unusual association between ITP and interstitial pneumonia, suggesting that a lung injury similar to TRALI is involved in acute and recurrent ITP. METHODS: We have identified patients with ITP who developed interstitial pneumonia during the course of ITP. We reviewed their charts and analyzed their clinical courses of ITP and interstitial lung diseases. Laboratory tests and chest X ray or CAT scans were reviewed. The laboratory study included CBC, platelets and platelets activation was measured by PMP (platelet microparticles), expression of CD62p flowcytometrically. RESULTS: We have identified 6 patients with ITP who developed interstitial pneumonia during the course of ITP. In two of six, interstitial pneumonia was detected at the presentation of acute ITP. ITP was severe with platelet counts less than 10.000. Interstitial pneumonia was discovered incidentally by chest X ray and confirmed by CAT scans. A mild symptom of dyspnea was detected in careful examination. One underwent lung biopsy which showed findings consistent with brochiolitis obliterans organizing pneumonia. Repeated CAT scans in 1–3 months revealed marked improvement but residual interstitial infiltrates still persisted. Four others had a long standing chronic ITP with clinical courses characterized by frequent relapses in spite of surgical and medical therapy. Four of six patients had splenectomy. Interstitial lung diseases were detected at the time of a severe relapse with platelet counts of less than 20.000. One patient underwent chromium labeled platelet sequestration study which revealed rapid sequestration of platelets in the lung. Interstitial infiltrates improved following improvement of ITP but two progressed to interstitial pulmonary fibrosis. CD62P measured by flowcytometry was very high in all 3 patients tested, indicating persisting platelet activation in this clinical setting. SUMMARY: We report interstitial pneumonia developing in 6 patients with ITP. Clinically all were asymptomatic and detection of interstitial pneumonia was incidental radiology finding. A mild symptom of exertional dyspnea was present in careful investigation. Chest X ray or CT scans showed nonspecific interstitial infiltrates and showed an overall improvement within months but residual infiltrates persisted. Two progressed to pulmonary fibrosis. We suggest that platelets are sequestered and destroyed in the lung in some patients with ITP, to generate cytokines and lipid mediators that lead to a nonspecific interstitial lung disease.

Author(s):  
Christopher H. Fanta

This Chest X-Ray Refresher is organized as a game. For each of the three topics to be discussed, we offer four chest x-rays and four clinical histories. The order of each set is random. The exercise asks that you consider the clues in the history and the findings on chest x-ray to match the history with the x-ray. In many instances, the combination will suggest a diagnosis or a limited differential of diagnostic possibilities. The three topics to be discussed are hemoptysis, chronic interstitial lung diseases, and obstructive lung diseases.


Chest Imaging ◽  
2019 ◽  
pp. 453-457
Author(s):  
Cylen Javidan-Nejad

Idiopathic pulmonary fibrosis (IPF) represents one of the most common chronic interstitial lung diseases. Usual interstitial pneumonia (UIP) is the pathologic diagnosis of IPF and can be diagnosed when honeycombing is present with a basilar and peripheral predominance and findings not typical of UIP are absent. In the current era, when a diagnosis of UIP is made with confidence on HRCT, biopsy can be avoided. Yet, one must be familiar with mimics of UIP/IPF (most notably pulmonary edema superimposed on emphysema) to avoid confusion misdiagnosis. Radiologists must also be familiar with potential complications of UIP including progression, infection, accelerated fibrosis (which can be lethal) and primary lung cancer (which has an increased incidence in UIP).


2017 ◽  
Vol 9 (1) ◽  
pp. e2017060
Author(s):  
George J Kontoghiorghes

Two separate episodes of transfusion-related acute lung injury (TRALI) in thalassaemia patients caused by red blood cell transfusions from the same multiparous blood donor are reported. Both cases had the same symptomatology and occurred 10-60 minutes of transfusion. The patients presented dyspnea, sweating, fatigue, dizziness, fever, and sense of losing consciousness. The chest x-ray showed a pulmonary oedema-like picture with both lungs filled with fluid. The patients were treated in the intensive therapy unit. They were weaned off the ventilator and discharged following hospitalization 7 and 9 days respectively. The TRALI syndrome was diagnosed to be associated with HLA-specific donor antibodies against mismatched HLA-antigens of the transfused patients. Haemovigilance improvements are essential for reducing the morbidity and mortality in transfused patients. Blood from multiparous donors should be tested for the presence of IgG HLA-Class I and –Class II antibodies before being transfused in thalassaemia and other chronically transfused patients.


Author(s):  
Javier Alcazar-Castro ◽  
Alejandro Zarate-Aspiros ◽  
Elias Andrade-Cuellar ◽  
Brenda Alvarez-Perez ◽  
Alan I. Valderrama-Treviño ◽  
...  

Acute pulmonary damage caused by transfusion is characterized by the sudden onset of respiratory distress in newly transfused patients within 6 hours after the transfusion, bilateral infiltrative changes in chest X-ray, PaO2/FIO2 <300 mmHg, absence of other risk factors for acute lung injury and absence of signs suggesting cardiogenic origin of pulmonary edema. Being one of the most serious complications of blood transfusion, plasma is the most involved factor, although all blood components can cause it, and is caused by antigen reactions/leukocyte antibody and lipid activity with ability to modify the biological response on primitive leukocytes. The diagnosis is based on the integration of clinical, radiological and gasometric elements, ruling out the rest of the possible causes of acute lung injury. Its differential diagnosis should include hemodynamic overload, anaphylactic reaction, bacterial contamination of transfused blood products and transfusion hemolytic reaction. The treatment is supportive measures based on the needs and does not differ from the treatment of acute lung injury secondary to other etiologies, severe cases require endotracheal intubation and mechanical ventilation while the non-severe can be managed with oxygen therapy.


2021 ◽  
pp. S353-S366
Author(s):  
D Mokra

Acute lung injury is characterized by acute respiratory insufficiency with tachypnea, cyanosis refractory to oxygen, decreased lung compliance, and diffuse alveolar infiltrates on chest X-ray. The 1994 American-European Consensus Conference defined “acute respiratory distress syndrome, ARDS” by acute onset after a known trigger, severe hypoxemia defined by PaO2/FiO2≤200 mm Hg, bilateral infiltrates on chest X-ray, and absence of cardiogenic edema. Milder form of the syndrome with PaO2/FiO2 between 200-300 mm Hg was named „acute lung injury, ALI“. Berlin Classification in 2012 defined three categories of ARDS according to hypoxemia (mild, moderate, and severe), and the term “acute lung injury” was assigned for general description or for animal models. ALI/ARDS can originate from direct lung triggers such as pneumonia or aspiration, or from extrapulmonary reasons such as sepsis or trauma. Despite growing understanding the ARDS pathophysiology, efficacy of standard treatments, such as lung protective ventilation, prone positioning, and neuromuscular blockers, is often limited. However, there is an increasing evidence that direct and indirect forms of ARDS may differ not only in the manifestations of alterations, but also in the response to treatment. Thus, individualized treatment according to ARDS subtypes may enhance the efficacy of given treatment and improve the survival of patients.


2021 ◽  
Vol 64 (4) ◽  
pp. 277-285
Author(s):  
Jongmin Lee

Interstitial lung diseases (ILD) refers to a large and heterogenous group of parenchymal lung disorders. It is difficult to diagnose and classify ILD. Nevertheless, accurate diagnosis of ILD is crucial for appropriate treatment selection and prediction of prognosis. Idiopathic pulmonary fibrosis (IPF), the most severe of the chronic forms of ILD, is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause. In addition to IPF, a subset of patients with ILD may develop progressive fibrotic changes in lungs. As pulmonary fibrosis progresses, lung function gradually deteriorates and respiratory symptoms worsen; besides, quality of life is also impaired. Progressive fibrosis is also associated with limited response to immunomodulatory thrapies and, potentially, early death. A progressive fibrosing phenotype of ILD (PF-ILD), a subtype of ILD, shows morphological similarities, common underlying pathophysiologic mechanisms, and consistently progressive worsening. PF-ILD include idiopathic nonspecific interstitial pneumonia, unclassifiable idiopathic interstitial pneumonia, autoimmune ILD, chronic sarcoidosis, chronic hypersensitivity pneumonitis and environmental lung diseases. Antifibrotic agents pirfenidone and nintedanib have showed positive results not only for IPF but also for PF-ILD. Immunosuppressive therapy can be used for some types of PF-ILD. If a patient with PF-ILD does not respond to conventional treatment, lung transplantation may be a treatment option. Clinical trials on the treatment of PF-ILD are actively underway. Therefore, over the course of the next several years, major advances in PF-ILD treatment can be expected.


Author(s):  
Poonam Vohra ◽  
Harsumeet S. Sidhu

Background: Diffuse lung diseases describe a heterogeneous group of disorders of the lower respiratory tract characterized by inflammation and derangement of the interstitium and loss of functional alveolar units. The disease is not restricted to the interstitium only, as it involves epithelial, endothelial and mesenchymal cells with the disease process extending into the alveoli, acini and bronchioles. Thus, the entire pulmonary parenchyma is involved. The objective of the study was to evaluate diffuse lung diseases by high resolution computed tomography of chest.Methods: A cross-sectional observational study was done in 30 patients. Adult patients of either sex of age group 18 and above showing reticular opacities on chest X-ray and those patients who were incidentally diagnosed as cases of diffuse lung diseases on HRCT chest were included in present study.Results: Reticular opacities were the most common roentgenographic finding followed by reticulonodular opacities. On HRCT, intra and interlobular septal thickening was the most common finding in Idiopathic interstitial pneumonia (usual interstitial pneumonia).Conclusions: High resolution computed tomography (HRCT) is superior to the plain chest X-ray for early detection and confirmation of suspected diffuse lung diseases. In addition, HRCT allows better assessment of the extent and distribution of disease, and it is especially useful in the investigation of patients with a normal chest radiograph. Coexisting disease is often best recognized on HRCT scanning.


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