Cause Of Alveolar Hemorrhage Research Articles

Diffuse Alveolar Hemorrhage in Hematopoietic Cell Transplantation.

Diffuse alveolar hemorrhage (DAH) is a morbid syndrome that occurs after autologous and allogeneic hematopoietic cell transplantation in children and adults. DAH manifests most often in the first few weeks following transplantation. It presents with pneumonia-like symptoms and acute respiratory failure, often requiring high levels of oxygen supplementation or mechanical ventilatory support. Hemoptysis is variably present. Chest radiographs typically feature widespread alveolar filling, sometimes with peripheral sparing and pleural effusions. The diagnosis is suspected when serial bronchoalveolar lavages return increasingly bloody fluid. DAH is differentiated from infectious causes of alveolar hemorrhage when extensive microbiological testing reveals no pulmonary pathogens. The cause is poorly understood, though preclinical and clinical studies implicate pretransplant conditioning regimens, particularly those using high doses of total-body-irradiation, acute graft-versus-host disease (GVHD), medications used to prevent GVHD, and other factors. Treatment consists of supportive care, systemic corticosteroids, platelet transfusions, and sometimes includes antifibrinolytic drugs and topical procoagulant factors. Therapeutic blockade of tumor necrosis factor-α showed promise in observational studies, but its benefit for DAH remains uncertain after small clinical trials. Even with these treatments, mortality from progression and relapse is high. Future investigational therapies could target the vascular endothelial cell biology theorized to contribute to alveolar bleeding and pathways that contribute to susceptibility, inflammation, cellular resilience, and tissue repair. This review will help clinicians navigate through the limited evidence to diagnose and treat DAH, counsel patients and families, and plan for future research.

End-Stage Renal Disease, Autoimmune Hepatitis, Alveolar Hemorrhage and ANCA Vasculitis as a Complication of Chronic Hepatitis B Virus: An Unusual Case Report

Background: End stage renal disease, autoimmune hepatitis and alveolar hemorrhage have rarely been reported as the complications of hepatitis B virus infection that can potentially be triggered by an antibody-mediated vasculitis as a result of a viral infection. The following was a case report detailing a patient presenting with all three of the above presentations who was cytoplasmic antineutrophil cytoplasmic antibody-positive and a chronic carrier of hepatitis B virus. Case presentation: A 52-year-old Malagasy woman presented in our intensive care unit for acute respiratory failure with end-stage renal disease and deterioration in general condition. She was managed with steroids, non-invasive ventilation, and hemodialysis. Laboratory test showed elevated levels of serum creatinine and liver enzymes. An extensive workup was done to find the etiology of elevated liver enzymes. Only blood work that came back positive was for chronic inactive hepatitis virus B and positive autoantibody suggestive of autoimmune hepatitis. A computed tomography scan of her lung revealed features suggestive of bilateral alveolar hemorrhage. Workup for other causes of vasculitis, including anti-neutrophil cytoplasmic antibodies (ANCA) was positive for c-ANCA antibody to proteinase 3. Medical investigations allowed excluding other infectious diseases, malignancies and other rheumatic and vasculitis affections. Administration of lamivudine® orally was done. The patient was followed for more than one year and there were no other new systematic symptoms. Conclusion: This case report showed the possibility of hepatitis B virus-induced vasculitis as the cause of alveolar hemorrhage, autoimmune hepatitis, and end stage renal disease. The origin of these symptoms was attributed to immune complex-mediated vasculitis induced by antineutrophil cytoplasmic antibody.

O18-5 - 2 Case Reports of Successful Treatment by Central ECMO with LV Venting for Severe Heart Failure Complicated with Alveolar Hemorrhage

Severe heart failure patients are sometimes complicated with alveolar hemorrhage due to pulmonary congestion. Patients with alveolar hemorrhage have high pulmonary vascular resistance, so LVAD attachment induces relative right heart failure which may require right heart system assist. In such cases, hemodynamic management becomes very difficult. With high pulmonary perfusion as well as intensive anti-coagulation therapy, alveolar hemorrhage might worsen. Furthermore, it is difficult to tell whether the cause of alveolar hemorrhage is pulmonary congestion or other diseases like organized pneumonia. Our present strategy for particularly severe heart failure is as follows: we adopt central ECMO support with RA drainage and ascending aorta perfusion to get adequate circulatory flow support. At the same time, LV venting from the apex is also used to release pulmonary congestion, which leads to recover pulmonary function, and reduce pulmonary vascular resistance. And as a next step, we aim to maintain hemodynamics by supporting left heart system only. Recently, we experienced 2 severe heart failure cases complicated with alveolar hemorrhage whose etiologies were hard to differentiate, which were successfully treated according to our strategy. Here, we are going to report these 2 cases with some discussions.

A Rare Cause of Childhood Anaemia: Idiopathic Pulmonary Hemosiderosis

Often seen in childhood, idiopathic pulmonary hemosiderosis (IPH) is a rare cause of alveolar haemorrhage with unknown etiology. It is characterised by recurrent hemoptysis, iron deficiency anaemia, and diffuse parenchymal infiltration in chest radiography (1). The incidence rate of IPH in children is between 0,24 and 1,26 in a million and this explains the very few number of reported cases in the literature (2).

Severe Respiratory Distress in a Child with Pulmonary Idiopathic Hemosiderosis Initially Presenting with Iron-Deficiency Anemia.

Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of alveolar hemorrhage in children but should be considered in children with anemia of unknown origin who develop respiratory complications. It is commonly characterized by the triad of recurrent hemoptysis, diffuse parenchymal infiltrates, and iron-deficiency anemia. Pathogenesis is unclear and diagnosis may be difficult along with a variable clinical course. A 6-year-old boy was admitted to the hospital with a severe iron-deficiency anemia, but he later developed severe acute respiratory failure and hemoptysis requiring intubation and mechanical ventilation. The suspicion of IPH led to the use of immunosuppressive therapy with high dose of corticosteroids with rapid improvement in clinical condition and discharge from hospital.

New insights into pediatric idiopathic pulmonary hemosiderosis: the French RespiRare® cohort

BackgroundIdiopathic pulmonary hemosiderosis (IPH) is a rare cause of alveolar hemorrhage in children and its pathophysiology remains obscure. Classically, diagnosis is based on a triad including hemoptysis, diffuse parenchymal infiltrates on chest X-rays, and iron-deficiency anemia. We present the French pediatric cohort of IPH collected through the French Reference Center for Rare Lung Diseases (RespiRare®, http://www.respirare.fr).MethodsSince 2008, a national network/web-linked RespiRare® database has been set up in 12 French pediatric respiratory centres. It is structured as a medical recording tool with extended disease-specific datasets containing clinical information relevant to all forms of rare lung diseases including IPH.ResultsWe identified 25 reported cases of IPH in children from the database (20 females and 5 males). Among them, 5 presented with Down syndrome. Upon diagnosis, median age was 4.3 [0.8-14.0] yrs, and the main manifestations were: dyspnea (n = 17, 68%), anemia (n = 16, 64%), cough (n = 12, 48%), febrile pneumonia (n = 11, 44%) and hemoptysis (n = 11, 44%). Half of the patients demonstrated diffuse parenchymal infiltrates on chest imaging, and diagnosis was ascertained either by broncho-alveolar lavage indicating the presence of hemosiderin-laden macrophages (19/25 cases), or lung biopsy (6/25). In screened patients, initial auto-immune screening revealed positive antineutrophilic cytoplasmic antibodies (ANCA) (n = 6, 40%), antinuclear antibodies (ANA) (n = 5, 45%) and specific coeliac disease antibodies (n = 4, 28%). All the patients were initially treated by corticosteroids. In 13 cases, immunosuppressants were introduced due to corticoresistance and/or major side effects. Median length of follow-up was 5.5 yrs, with a satisfactory respiratory outcome in 23/25 patients. One patient developed severe pulmonary fibrosis, and another with Down syndrome died as a result of severe pulmonary hemorrhage.ConclusionThe present cohort provides substantial information on clinical expression and outcomes of pediatric IPH. Analysis of potential contributors supports a role of auto-immunity in disease development and highlights the importance of genetic factors.

Idiopathic pulmonary hemorrhage: morphology and differential diagnosis. Case report

Idiopathic pulmonary hemorrhage (IPH) is a rare cause of alveolar hemorrhage (AH) with unknown etiology that primarily affects children. The process has a variable clinical progression, and its diagnosis is established after excluding all causes of AH. Herein, the authors report a case of IPH in an adult male patient referring cough and hemoptysis. The conventional radiography computed tomography imaging identified zones of consolidation and areas of ground-glass attenuation in the lower lobes and lingula. Forced spirometry, bronchoscopy, and laboratorial tests yielded normal results. Several alveolar hemosiderin-laden macrophages were identified on bronchoalveolar lavage and lung biopsy. Thus, the histopathological findings associated with clinical data were compatible with IPH.

Alveolar hemorrhage and kidney disease: Characteristics and therapy

Anti-neutrophil cytoplasmic antibody-associated vasculitis and Goodpasture's glomerular basement membrane disease are the most common causes of diffuse alveolar hemorrhage, a life-threatening disease. Systemic lupus erythematosus and the antiphospholipid syndrome are also causes of alveolar hemorrhage. We retrospectively reviewed 15 cases of diffuse alveolar hemorrhage (DAH) associated with renal diseases. Diagnosis of DAH was based on the presence of bloody bronchoalveolar lavage fluid. There were three men and 12 women, with a mean age of 50.5 years (extremes: 24-74 years). Proteinuria and hematuria were observed, respectively, in 15 and 14 cases. Six patients revealed arterial hypertension. Crescentic glomerulonephritis was diagnosed with kidney biopsies in ten cases. The etiology of renal disease was microscopic polyangiitis (MPA) in seven cases, Wegener disease in four cases, systemic lupus erythematous in one case, cryoglobulinemia in one case, myeloma in one case and propyl-thiouracil-induced MPA in one case. Hemoptysis occurred in 14 cases. The mean serum level of hemoglobin was 7.1 g/dL (5.1-10 g/dL). The mean serum creatinine concentration was 7.07 mg/dL (2.4-13.7 mg/dL). Gas exchange was severely compromised, with an oxygenation index <80 mmHg in 14 patients and <60 mmHg in seven patients. Bronchoalveolar lavage was performed in 11 cases, and had positive findings for hemorrhage in all. Methylprednisolone pulses and cyclophosphamide were used in 14 patients. Plasmapheresis was performed in three cases. One patient received cycles of Dexamethasome-Melphalan. Three patients died as a result of DAH. The mortality rate in our study was 20%.

Management of Alveolar Hemorrhage in Lung Vasculitides

Alveolar hemorrhage (AH) is an important pulmonary manifestation of small vessel vasculitis because severe presentations are the most common vasculitic cause of early death. Renal vasculitis is usually present with AH; the combination is known as pulmonary-renal syndrome. Early diagnosis and intensive therapy are of particular importance to reduce early mortality and improve longer-term outcomes. The commonest immune-mediated cause of AH is anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) (80%), with other vasculitides, including systemic lupus erythematosus and anti-glomerular basement membrane disease accounting for 20%. One quarter of AAV patients develop AH, which when mild is associated with a good outcome, but mortality rises to 50% for cases with respiratory failure requiring ventilator support. The prognosis of AH in the other vasculitides is generally favorable, but cases are rare and experience is limited. Treatment follows similar regimens to those for other AAV presentations, although when severe there is widespread use of parenteral glucocorticoids together with plasma exchange. These interventions have developed empirically supported by a theoretical rationale but have not been validated by randomized clinical trials. Sepsis and cardiovascular and thromboembolic events are important early complications. and long-term follow-up is required to monitor for and prevent relapse and manage disease-related damage. A minority of cases develop on a background of pulmonary fibrosis, or progressive pulmonary fibrosis develops after vasculitis has gone into remission.

Hémorragie intra-alvéolaire : présentation rare du syndrome d’embolie graisseuse

Fat embolism syndrome is a severe complication of long bone fractures, corresponding to the obstruction of small vessels by microdroplets of fat, originating from medulla ossium. Pulmonary involvement, present in 90% cases, makes the severity of the disease.We report the case of a 22-year-old man who presented, two days after industrial accident causing an opened tibial fracture, acute dyspnea with hemoptysis. Angio-CT-scan didn't show any proximal vascular obstruction, but parenchymal sections showed diffuse, bilateral and multifocal hyperdensities predominating at the periphery. Broncho-alveolar lavage brought a hemorrhagic liquid, with a high macrophage content and lipid inclusions in macrophages. Exams for the etiologic diagnosis of intra-alveolar hemorrhage were negative: renal function, 24-hour proteinuria, antinuclear antibodies, antineutrophil cytoplasmic antibodies. The diagnosis of intra-alveolar hemorrhage secondary to fat embolism was established. The outcome was spontaneously favorable.The occurrence of intra-alveolar hemorrhage in the course fat embolism is rarely reported. Its pathogenic mechanisms are not understood. It is mandatory to eliminate the other causes of alveolar hemorrhage before holding the diagnosis of fat embolism. Treatment is only symptomatic, based on respiratory reanimation.

Hemorragia alveolar difusa: causas y desenlaces en un instituto de tercer nivel

Objective To identify the most common causes of diffuse alveolar hemorrhage (DAH) and the evolution of cases during hospitalization. Patients and methods A review of cases diagnosed with DAH; the diagnoses were classified according to existing criteria and the progression of the cases was determined. Results We identified 17 cases of DAH, with the leading cause being ANCA associated vasculitis (41% of cases), followed by cases secondary to drugs (18%). In 35% of the cases, there was a failure in identifying an etiology. Six patients died (35%), the only factor associated with mortality was male gender 5/6 vs 3/11, p=0.05. Conclusions The most frequent cause of alveolar hemorrhage was ANCA associated vasculitis. The mortality in DAH is about 35%, males seem to have a worse prognosis.

Diffuse alveolar hemorrhage: Causes and outcomes in a referral center

ObjectiveTo identify the most common causes of diffuse alveolar hemorrhage (DAH) and the evolution of cases during hospitalization. Patients and methodsA review of cases diagnosed with DAH; the diagnoses were classified according to existing criteria and the progression of the cases was determined. ResultsWe identified 17 cases of DAH, with the leading cause being ANCA associated vasculitis (41% of cases), followed by cases secondary to drugs (18%). In 35% of the cases, there was a failure in identifying an etiology. Six patients died (35%), the only factor associated with mortality was male gender 5/6 vs 3/11, P=.05. ConclusionsThe most frequent cause of alveolar hemorrhage was ANCA associated vasculitis. The mortality in DAH is about 35%, males seem to have a worse prognosis.

Inhalant Abuse

An 18-year-old man presented to a community emergency department with increasing shortness of breath and fever. His condition was diagnosed, and he was treated as an inpatient for bilateral pneumonia associated with hypoxemia. When his condition became worse, he acknowledged to deliberate inhalation of keyboard cleaner and to having hemoptysis. Before his death on hospital day 11, known causes of alveolar hemorrhage were excluded. We postulated a cause-and-effect relationship, adding alveolar hemorrhage to the known complications of inhalant abuse.

An unusual cause of alveolar hemorrhage post hematopoietic stem cell transplantation: A case report

BackgroundHematopoietic stem cell transplantation is being increasingly used in cancer therapy. Diffuse alveolar hemorrhage, an early complication of stem cell transplant, results from bacterial, viral and fungal infections, coagulopathy, and engraftment syndrome, or can be idiopathic. Diffuse alveolar hemorrhage associated with Strongyloides stercoralis hyperinfection in stem cell transplant patients has been rarely reported.Case presentationWe describe an unusual cause of alveolar hemorrhage post hematopoietic stem cell transplant due to Strongyloides hyperinfection. Therapy with parenteral ivermectin and thiabendazole was initiated but the patient deteriorated and died of respiratory failure and septic shock.ConclusionStrongyloides stercoralis hyperinfection is an unusual cause of alveolar hemorrhage early after hematopoietic stem cell transplant with very high mortality.

A Case of Microscopic Polyangiitis with Diffuse Alveolar Hemorrhage

Diffuse alveolar hemorrhage is a rare but serious and frequently life-threatening complication of a variety of conditions. The first goal in the management of patients with diffuse alveolar hemorrhage is to achieve or preserve stability of the respiratory status. Subsequently, the differential diagnosis is aimed at the identification of a remediable cause of the alveolar hemorrhage. The most common causes of diffuse alveolar hemorrhage with glomerulonephritis are microscopic polyangiitis and Wegener's granulomatosis, followed by Goodpasture syndrome and systemic lupus erythematosus. Microscopic polyangiitis (MPA) is a distinct systemic small vessle vasculitis affecting small sized vessels with few or no immune deposits and with no granulomatosus inflammation. The disease may involve multiple organs such as kidney, lung, skin, joint, muscle, gastrointestinal tract, eye, and nervous system. MPA is strongly associated with antineutrophil cytoplasmic autoantibody (ANCA) that is a useful serological diagnostic marker for the most common form of necrotizing vasculitis. Our report concerns a case of microscopic polyangiitis with diffuse alveolar hemorrhage in a 54-year-old man. He was admitted to our hospital due to dyspnea upon exertion and recurrent hemoptysis. Laboratory findings showed hematuria, proteinuria and deterioration of renal function. In the chest CT scan, diffuse ground glass appearance was seen in both lower lungs. A lung biopsy revealed small vessel vasculitis with intraalveolar hemorrhage and showed a positive reaction to against perinuclear ANCA. The patient was treated with prednisolone and cyclophosphamide. Chest infiltration decreased and hemoptysis and hypoxia improved. He is still being followed up in our hospital with a low dose of prednisolone.