Diffuse Alveolar Hemorrhage Following Pirfenidone Initiation

2019 ◽  
Vol 33 (4) ◽  
pp. 548-552 ◽  
Author(s):  
Stacey K. Dull ◽  
Nikhil Jagan ◽  
Douglas R. Moore ◽  
Zachary S. DePew ◽  
Lee E. Morrow ◽  
...  

Introduction Diffuse alveolar hemorrhage (DAH) is bleeding into the alveolar space of the lungs. Pirfenidone is an antifibrotic agent that is approved for the treatment of idiopathic pulmonary fibrosis (IPF). The most commonly reported side effects include gastrointestinal and skin-related events. We present 3 cases of hemoptysis and DAH among patients on pirfenidone therapy for IPF. Case Summaries An 88-year-old female, a 75-year-old male, and a 73-year-old male all with IPF on pirfenidone presented with hemoptysis and chest computed tomography (CT) findings of usual interstitial pneumonia (UIP) with superimposed opacities. In 2 patients, DAH was confirmed with bronchoscopy. Corticosteroids were initiated and pirfenidone discontinued in all patients, and 2 patients improved while the third continued to deteriorate. Nintedanib was initiated in the remaining 2 patients at follow-up visit with no further issues. Discussion IPF is a chronic, progressive, fibrotic interstitial lung disease (ILD) which appears to be increasing in the United States and has a relatively short survival. Nintedanib and pirfenidone were the first Food and Drug Administration (FDA)-approved agents for the treatment of IPF in October 2014. We present 3 cases of DAH in patients with IPF receiving pirfenidone. Symptoms occurred within 2 months of pirfenidone initiation and resolved with discontinuation of pirfenidone and initiation of systemic corticosteroids in 2 patients; however, one case was complicated by concomitant discontinuation of aspirin. The mechanism by which DAH occurred in our patients remains unclear. Conclusion We report the first cases of possible pirfenidone-induced DAH. Further studies are warranted to explore this reaction, but prescribers should be cognizant of this potential issue when choosing to prescribe pirfenidone.

2019 ◽  
Vol 2 (2) ◽  
pp. 229-233
Author(s):  
Ashesh Dhungana ◽  
Prajowl Shrestha

Introduction: Diffuse alveolar hemorrhage results from an accumulation of red blood cells into the alveolar space. Symptoms of alveolar hemorrhage are dyspnea, hemoptysis, anemia, diffuse pulmonary infiltrates and hypoxemic respiratory failure. Diagnosis is established by bronchoalveolar lavage and treatment includes a combination of high dose systemic corticosteroids, immunosuppressant and plasma exchange. The aim of this study is to evaluate the clinical radiological profile and laboratory findings and utility of bronchoalveolar lavage in the diagnosis of diffuse alveolar hemorrhage.Materials and Methods: In a retrospective review between February 2017 and December 2017, medical records of patients with a diagnosis of diffuse alveolar hemorrhage presenting at the National Academy of Medical Sciences, Kathmandu, Nepal, were analyzed. Clinical, radiology and laboratory results along with bronchoalveolar lavage results were extracted. Treatment received and clinical responses were evaluated.Results: A total of five patients were diagnosed to have diffuse alveolar hemorrhage based on bronchoalveolar lavage analysis. Three had hemorrhage secondary to Antineutrophil Cytoplasmic Antibody associated vasculitis, one had Systemic Lupus Erythematosus and the other Idiopathic Pulmonary Hemosiderosis. Renal involvement was present in three patients. All patients received systemic corticosteroids, three received Cyclophosphamide and one Rituximab for remission induction. Plasma exchange was done in two patients with severe hypoxemia. Of the five patients, four improved whereas one died.Conclusions: Diffuse alveolar hemorrhage presents with non-specific symptoms. Bronchoalveolar lavage is extremely useful to establish the diagnosis and exclude infections. Early initiation of immunosuppressant prevents respiratory failure and death.


2020 ◽  
Vol 4 (4) ◽  
pp. 537-539
Author(s):  
Gideon Logan ◽  
Ernesto Robalino ◽  
Tracy MacIntosh ◽  
Latha Ganti

Introduction: Drug overdose represents a growing reason for emergency department visits and hospitalizations in the United States. Co-ingestion of multiple substances is also on the rise, and toxidromes can be seen from any of multiple drugs in a single patient. Case Report: We present a case of diffuse alveolar hemorrhage secondary to cocaine abuse in a patient who was apneic and unresponsive after heroin overdose. The patient responded to supportive care and was discharged with complete return to physical and mental baseline. Conclusion: Clinicians must be vigilant for any number of concomitant toxidromes when a patient is brought in with complications following drug overdose.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1751.2-1751
Author(s):  
J. Zalc ◽  
G. Camille ◽  
B. Lisa ◽  
J. Chloé ◽  
B. Frédéric

Background:Osteoporosis is a public health issue. Lack of therapeutic compliance is often a problem in the treatment of osteoroposis, with potentially dramatic consequences. No studies have evaluated the observance of zoledronic acid infusion after 3 years, the time of the therapeutic reassessment.Objectives:The main objective of assessing the level of compliance was to evaluate the level of zoledronic acid infusion adherence at 1, 2 and 3 year periods, in a cohort of osteoporotic patients on discharge from Begin hospital, following treatment for fracture caused by low-energy trauma. The first infusion was prescribed by rheumatologists, with the following infusions to be prescribed by general practitioners.Methods:We performed a retrospective observational study initially conducted by written and telephone questionnaires on a population of patients hospitalized in the rheumatology department of HIA Bégin for an osteoporotic fracture. Data was collected between July 2015 and December 2018. A first letter, containing a stamped addressed envelope to the Bégin hospital for ease of reply, was sent to the patients selected for the study. The protocol had to be modified following a very low response rate, unaided by bad quality addresses. We then tried to contact the patients by phone 3 times and, if unable to reach them, we called their general practitioners on 3 occasions.Results:94 patients were initially selected. Every year, we retained within the study patients who had followed their annual zoledronic acid infusion protocol. Taking into account all 94 patients, adherence level for the first infusion was 41.4%, down to 29.7% for the second infusion and down to 12.8% for the third infusion.For those who had the first infusion performed, adherence level for the second infusion was 71.8%, down to 30.8% for the third infusion.Conclusion:The observance and follow-up of zoledronic acid infusion in France by general practitioners is not adequate. Follow-up measures on an annual basis by the rheumatologist could significantly improve adherence.References:[1]Curtis JR et al. Adherence with intravenous zoledronate and intravenous ibandronate in the United States Medicare population. Arthritis care & research. 2012;64:1054-60.[2]Jaleel A et al. Improving drug adherence in osteoporosis: an update on more recent studies. Therapeutic advances in musculoskeletal disease. 2018;10:141-9.Acknowledgments:NoneDisclosure of Interests:None declared


2021 ◽  
Vol 84 (4) ◽  
pp. 255-262
Author(s):  
Min Jung Kim ◽  
Kichul Shin

Microscopic polyangiitis (MPA) is an antineutrophil cytoplasmic antibody (ANCA)‒associated necrotizing vasculitis, which mainly affects small vessels in various organs, especially the lungs. The two key pulmonary manifestations, interstitial lung disease (ILD) and diffuse alveolar hemorrhage (DAH), increase the morbidity and death rate of patients with MPA. ILD is more common in MPA than in other ANCA-associated vasculitis subsets and is primarily associated with myeloperoxidase-ANCA. Unlike alveolar hemorrhage due to pulmonary capillaritis, ILD can initially manifest as isolated pulmonary fibrosis. Of note, its most frequent radiographic pattern is the usual interstitial pneumonia pattern, similar to the characteristic pattern seen in idiopathic pulmonary fibrosis. In this review we present the pathogenesis, clinical manifestations, and radiographic and histopathologic features of ILD and DAH in MPA. We also briefly summarize the outcome and therapeutic options for the two conditions.


PEDIATRICS ◽  
1974 ◽  
Vol 53 (3) ◽  
pp. 353-357
Author(s):  
Neil A. Holtzman ◽  
E. David Mellits ◽  
Clayton H. Kallman

The relationships between initial and follow-up blood phenylalanine concentration and between initial concentration and age were examined in infants with elevated screening tests. Data were provided by health departments and phenylketonuria (PKU) clinics. Among infants who attained levels of 20 mg/100 ml or more the following was observed: (1) one-fourth had only minimal elevations (to less than 10 mg/100 ml) on the initial screening test; (2) of those with minimal elevations 66% were screened on or before the third day of life; (3) most of those whose initial elevations were more than minimally elevated and most of those with minimal elevations who were screened early had follow-up phenylalanines of 30 mg/100 ml or more. In contrast, those with minimal initial elevations who were screened late were more likely to have follow-up phenylalanines between 20 and 30 mg/100 ml. They may represent a different form of PKU. The findings suggest that as long as most infants in the United States continue to be screened on or before 4 days of age, some infants with PKU will be missed.


2021 ◽  
Author(s):  
Jessica L. Bloom ◽  
Benjamin Frank ◽  
Jason P. Weinman ◽  
Csaba Galambos ◽  
Sean T. O’Leary ◽  
...  

Abstract Background: Respiratory conditions are the leading cause of hospitalization and death in children with Trisomy 21 (T21). Diffuse alveolar hemorrhage (DAH) occurs at higher frequency in children with T21; yet, it is not widely studied nor is there a standardized approach to diagnosis or management. The objective of this study was to identify children with T21 and DAH in order to understand contributing factors and identify opportunities to improve outcomes. We identified 5 children with T21 at a single institution with histology-proven DAH over 10 years and discuss their presentation, evaluation, management, and outcomes. We also reviewed the cases in the literature. Case Presentation: Patient 1 died at age seven due to secondary hemophagocytic lymphohistiocytosis. DAH was seen on autopsy. Patient 2 was a three-year-old with systemic-onset juvenile idiopathic arthritis diagnosed with DAH after presenting for hypoxia. Patient 3 was diagnosed with DAH at age nine after presenting with recurrent suspected pneumonia and aspiration. Patient 4 was diagnosed with DAH at age eight after presenting with pallor and fatigue. She had additional ICU admissions for DAH with infections. Patient 5 developed hemoptysis at age three and had recurrent DAH for ten years. Four patients responded positively to immune-modulation such as intravenous immunoglobulin, glucocorticoids, and rituximab. Of the 19 patients identified in the literature, only one was from the United States. The majority had anemia, respiratory distress, autoantibodies, and recurrences. A minority had hemoptysis. Idiopathic pulmonary hemosiderosis was the most common diagnosis. Almost all received glucocorticoids with or without additional immunosuppression. The majority of our patients and those in the literature had positive auto-antibodies such as anti-neutrophil cytoplasmic antibodies and anti-nuclear antigen antibodies. Diagnostic clues included respiratory distress, hypoxia, anemia, recurrent pneumonia, and/or ground glass opacities on imaging. We identified four contributors to DAH: structural lung abnormalities, pulmonary arterial hypertension, infection/aspiration, and autoimmune disease/immune dysregulation.Conclusion: These cases demonstrate the need for an increased index of suspicion for DAH in children with T21, enrich the understanding of risk factors, and highlight the favorable response to immunosuppressive therapies in this vulnerable population.


2021 ◽  
Vol 2021 ◽  
pp. 1-4
Author(s):  
Khizar Hamid ◽  
Swaminathan Perinkulam Sathyanarayanan ◽  
Touba Naim ◽  
Muhammad Hamza ◽  
Mirza Omer Mahmood Baig ◽  
...  

Hantavirus Cardiopulmonary Syndrome (HCPS) can occur after infection with Hantavirus which can occur by inhaling aerosolized rodent urine, feces, and saliva contaminated with the virus. It presents with the rapid development of pulmonary edema, respiratory failure, and cardiogenic shock with the hallmark being microvascular leakage. We report a patient with a history of alcohol abuse and recent exposure to mice and sick kittens who presented with cough with sputum production, shortness of breath, orthopnea, and new-onset lower extremity edema. Imaging revealed bilateral infiltrates more common on the left with an unremarkable echocardiogram. Testing for COVID-19, Human Immunodeficiency Virus (HIV), influenza, bacterial pneumonia including tuberculosis and methicillin-resistant Staphylococcus aureus (MRSA), aspergillosis, histoplasmosis, Blastomyces, and Coccidiodes was negative. Bronchoscopy and bronchoalveolar lavage revealed diffuse alveolar hemorrhage (DAH) and were negative for acid-fast bacilli and Nocardia cultures. He was further tested for Hantavirus, Q fever, leptospirosis, toxoplasmosis, and empiric treatment with doxycycline initiated. His Hantavirus IgM antibody came back positive. Human Hantavirus infection occurs after inhalation of infected rodent excreta; fortunately, human-to-human transmission has not been documented. HCPS most commonly occurs due to the Sin Nombre virus (SNV), has a case fatality rate of 50%, and is a notifiable disease in the United States. It has 3 distinct phases, prodromal, cardiopulmonary, and convalescent/recovery. The cardiopulmonary phase occurs from increased permeability of pulmonary capillaries and in severe cases can progress to cardiogenic shock. Diagnosis is based on the presence of IgM and IgG Hantavirus antibodies. Treatment is mainly supportive; however, patients are usually treated with broad-spectrum antibiotics while workup is underway. In animal models, ribavirin and favipiravir are only effective when administered in the prodromal phase. If suspicion of Hantavirus infection exists, early mobilization to the intensive care unit for treatment is recommended. Extracorporeal membrane oxygenation (ECMO) has been suggested to improve outcomes in severe HCPS with refractory shock.


2021 ◽  
Vol 02 ◽  
Author(s):  
Trilok Chand ◽  
Georgie Thomas ◽  
M. U. Khan

Background: Pulmonary tuberculosis (TB) and diffuse alveolar hemorrhage (DAH) have some commonalities in several parts of the world. However, acute hemoptysis with diffuse alveolar shadows while a patient is on anticoagulant and antiplatelet therapy for a specific reason suggests DAH over TB. Case Presentation: In this case, a patient was presented with acute respiratory symptoms with hypoxia and bilateral alveolar shadows. He was treated for DAH at the initial encounter. However, on follow-up, he was confirmed having active pulmonary TB based on sputum acid-fast-bacilli culture. Conclusion: He was successfully treated with standard first-line anti-tubercular therapy and was subsequently declared cured. Pulmonary DAH with TB, in the absence of an underlying autoimmune disorder, is rare.


2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Jessica L. Bloom ◽  
Benjamin Frank ◽  
Jason P. Weinman ◽  
Csaba Galambos ◽  
Sean T. O’Leary ◽  
...  

Abstract Background Respiratory conditions are the leading cause of hospitalization and death in children with Trisomy 21 (T21). Diffuse alveolar hemorrhage (DAH) occurs at higher frequency in children with T21; yet, it is not widely studied nor is there a standardized approach to diagnosis or management. The objective of this study was to identify children with T21 and DAH in order to understand contributing factors and identify opportunities to improve outcomes. We identified 5 children with T21 at a single institution with histology-proven DAH over 10 years and discuss their presentation, evaluation, management, and outcomes. We also reviewed the cases in the literature. Case presentation Patient 1 died at age seven due to secondary hemophagocytic lymphohistiocytosis. DAH was seen on autopsy. Patient 2 was a three-year-old with systemic-onset juvenile idiopathic arthritis diagnosed with DAH after presenting for hypoxia. Patient 3 was diagnosed with DAH at age nine after presenting with recurrent suspected pneumonia and aspiration. Patient 4 was diagnosed with DAH at age eight after presenting with pallor and fatigue. She had additional ICU admissions for DAH with infections. Patient 5 developed hemoptysis at age three and had recurrent DAH for 10 years. Four patients responded positively to immune-modulation such as intravenous immunoglobulin, glucocorticoids, and rituximab. Of the 19 patients identified in the literature, only one was from the United States. The majority had anemia, respiratory distress, autoantibodies, and recurrences. Very few patients had hemoptysis. Idiopathic pulmonary hemosiderosis was the most common diagnosis. Almost all received glucocorticoids with or without additional immunosuppression. The majority of our patients and those in the literature had positive auto-antibodies such as anti-neutrophil cytoplasmic antibodies and anti-nuclear antigen antibodies. Diagnostic clues included respiratory distress, hypoxia, anemia, recurrent pneumonia, and/or ground glass opacities on imaging. We identified four contributors to DAH: structural lung abnormalities, pulmonary arterial hypertension, infection/aspiration, and autoimmune disease/immune dysregulation. Conclusion These cases demonstrate the need for an increased index of suspicion for DAH in children with T21, particularly given the low frequency of hemoptysis at presentation, enrich the understanding of risk factors, and highlight the favorable response to immunosuppressive therapies in this vulnerable population.


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