scholarly journals Diffuse Alveolar Haemorrhage: A Single Centre Experience

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.

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 5761-5761 ◽  
Author(s):  
Ayed O Ayed ◽  
Jan S Moreb ◽  
Jack W. Hsu ◽  
John W Hiemenz ◽  
John R Wingard ◽  
...  

Abstract Background: Bortezomib, a proteasome inhibitor, is frequently used in treatment of patients with multiple myeloma (MM). Bortezomib is generally well tolerated with gastrointestinal symptoms and peripheral neuropathy being the most common adverse effects. Here we report cases of severe diffuse alveolar hemorrhage (DAH) associated with bortezomib administration. Methods and Results: We identified 3 cases of severe DAH that were associated with bortezomib administration in MM patients treated at our institution between 2010 and 2014 (Table 1). All 3 patients presented with fever and worsening hypoxia shortly after initiation of bortezomib therapy and later developed progressive respiratory failure due to DAH. None of 3 patients had any respiratory symptoms or preexisting pulmonary conditions prior to bortezomib initiation. In one patient respiratory symptoms developed after 4 doses of bortezomib, improved off therapy, however rapidly progressed to a respiratory failure with a subsequent bortezomib treatment. Imaging studies showed diffuse areas of ground-glass opacities, apparent prominence of segmental and subsegmental bronchi and interlobular septal thickening. Infectious workup, which included bronchoscopy with bronchoalveolar lavage and respiratory viral studies, was completely negative in all 3 patients. All patients received supportive care, empiric broad-spectrum antimicrobial therapy and high-dose glucocorticoids, which were started within 4 days of hospital admission. Two patients died from complications of DAH and 1 patient recovered without pulmonary sequelae. Conclusion: Bortezomib-associated DAH is a rare, but serious and potentially fatal adverse event. Pathogenesis remains unclear and is likely related to impaired regulation of the inflammatory response. Early recognition of bortezomib associated DAH is essential for immediate discontinuation of the drug and prompt initiation of high dose steroids. More reports are needed to provide further insights about the pathogenesis and most optimal management of bortezomib induced DAH. Abstract 5761. Table 1: Patients who developed DAH after bortezomib administration Pt Age/ Gender/Race Smoker Sx Bor dose Bor schedule # of Bor doses Time of onset after last bor dose (days) Platelet count at the onset (103/mm3) Imaging/BAL findings Time to initiation of high dose steroids Outcome 1 67/M/C Never SOB, fever 1.3 mg/m2 IV/2Xwk 8 5 157 Bilateral infiltrates and GGO/DAH 2 Death 2 72/M/C Yes Fever, cough 1.3 mg/m2 IV/2Xwk 2 3 108 Bilateral infiltrates/DAH 4 Recovery 3 55/M/C Yes Fever, chills 1.5 mg/m2 IV/2Xwk 1 2 144 Bilateral diffuse airspace disease/DAH 4 Death Abbreviations: Pt, patient; M, male; C, Caucasian; Sx, symptoms; Bor, bortezomib; BAL, broncho-alveolar lavage; GGO, ground-glass opacities. Disclosures No relevant conflicts of interest to declare.


Author(s):  
LJ Baxter ◽  
S Chen ◽  
JM Burton

Background: Longitudinally extensive transverse myelitis (LETM) is a demyelinating condition that is associated with diseases such as neuromyelitis optica spectrum disorder (NMOSD), acute disseminated encephalomyelitis, collagen vascular disease, or can be idiopathic. LETM can be severe enough to cause quadraparesis, marked sensory dysfunction, and respiratory failure. Rarely, these patients are unresponsive to conventional immune therapy. Methods: We report two cases of severe LETM with acute development of quadraparesis and respiratory failure requiring intensive care admission and failure to respond to high-dose corticosteroids, plasma exchange, IVIg and rituximab. Disease cessation and ultimately, significant recovery, was achieved after an 8-day cyclophosphamide induction. Results: A 21 yo female with antibody positive NMOSD and a 19 yo male with idiopathic LETM remained quadraparetic and ventilator dependent with active MRIs despite multiple courses of intravenous methylprednisolone, plasma exchanges, and in the NMOSD patient, IVIg and a 4-week course of rituximab. Both patients ultimately improved significantly and are now ambulatory with subsequent cyclophosphamide induction. Conclusions: In patients with severe LETM of presumed immune origin, who fail to respond to corticosteroids and plasma exchange, cyclophosphamide induction should be considered. This agent provides a more robust immunosuppressive response and can be induced rapidly. Cyclophosphamide effects and supportive evidence are further discussed.


2019 ◽  
Vol 89 (2) ◽  
Author(s):  
Anshul Mittal ◽  
Jagdish Chander Suri ◽  
Shibdas Chakrabarti ◽  
Pranav Ish

It is uncommon for Systemic lupus erythematosus (SLE) to present with diffuse alveolar hemorrhage (DAH) as the initial presentation. To diagnose this in a young male with no renal involvement is further uncommon. We report a case of a 16-year-old boy, who presented with hemoptysis and was eventually diagnosed as DAH with underlying SLE. Treatment with steroids and immunosuppressant helped in rapid recovery from this potentially life-threatening condition. This case highlights the need of defining diagnostic criteria for SLE in patients presenting as DAH and formulating guidelines for treatment of the same, especially in absence of co-existing lupus nephritis.


2022 ◽  
Vol 20 ◽  
pp. 205873922110508
Author(s):  
Changgon Kim ◽  
Hyun-Sook Kim

Catastrophic antiphospholipid syndrome (CAPS) is a lethal disease that occurs suddenly and progresses to multi-organ failure. We present a case of CAPS successfully treated with the rituximab biosimilar CT-P10. A 38-year-old man was referred with a sustained fever and unexplained elevated creatinine levels. Cardiac arrest by ventricular fibrillation occurred upon arrival at the hospital. We diagnosed probable CAPS because of coronary thrombus, renal impairment, suspected diffuse alveolar hemorrhage, and positive anticardiolipin antibody immunoglobulin G. We performed percutaneous coronary intervention for the cardiac arrest, and treated him with extracorporeal membrane oxygenation, mechanical ventilation, and continuous renal replacement therapy. When CAPS was diagnosed, we administered CT-P10 after administering high-dose glucocorticoid. Our case suggests that the use of a rituximab biosimilar is economically efficient in the treatment of CAPS, as in other rheumatic diseases. The patient was cured without recurrence at the 2-year follow-up.


2012 ◽  
Vol 19 (4) ◽  
pp. 623-627
Author(s):  
Shin-ichiro Yoshida ◽  
Miyuki Tanabe ◽  
Yoshiki Masuda ◽  
Hitoshi Imaizumi ◽  
Naoya Yama ◽  
...  

2020 ◽  
Vol 31 (11) ◽  
pp. 2688-2704 ◽  
Author(s):  
Marta Casal Moura ◽  
Maria V. Irazabal ◽  
Alfonso Eirin ◽  
Ladan Zand ◽  
Sanjeev Sethi ◽  
...  

BackgroundTreatment of patients with ANCA-associated vasculitis (AAV) and severe renal involvement is not established. We describe outcomes in response to rituximab (RTX) versus cyclophosphamide (CYC) and plasma exchange (PLEX).MethodsA retrospective cohort study of MPO- or PR3-ANCA–positive patients with AAV (MPA and GPA) and severe kidney disease (eGFR <30 ml/min per 1.73 m2). Remission, relapse, ESKD and death after remission-induction with CYC or RTX, with or without the use of PLEX, were compared.ResultsOf 467 patients with active renal involvement, 251 had severe kidney disease. Patients received CYC (n=161) or RTX (n=64) for remission-induction, and 51 were also treated with PLEX. Predictors for ESKD and/or death at 18 months were eGFR <15 ml/min per 1.73 m2 at diagnosis (IRR 3.09 [95% CI 1.49 to 6.40], P=0.002), renal recovery (IRR 0.27 [95% CI 0.12 to 0.64], P=0.003) and renal remission at 6 months (IRR 0.40 [95% CI 0.18 to 0.90], P=0.027). RTX was comparable to CYC in remission-induction (BVAS/WG=0) at 6 months (IRR 1.37 [95% CI 0.91 to 2.08], P=0.132). Addition of PLEX showed no benefit on remission-induction at 6 months (IRR 0.73 [95% CI 0.44 to 1.22], P=0.230), the rate of ESKD and/or death at 18 months (IRR 1.05 [95% CI 0.51 to 2.18], P=0.891), progression to ESKD (IRR 1.06 [95% CI 0.50 to 2.25], P=0.887), and survival at 24 months (IRR 0.54 [95% CI 0.16 to 1.85], P=0.330).ConclusionsThe apparent benefits and risks of using CYC or RTX for the treatment of patients with AAV and severe kidney disease are balanced. The addition of PLEX to standard remission-induction therapy showed no benefit in our cohort. A randomized controlled trial is the only satisfactory means to evaluate efficacy of remission-induction treatments in AAV with severe renal involvement.


2017 ◽  
Vol 2017 ◽  
pp. 1-7 ◽  
Author(s):  
Gabriela Montes-Rivera ◽  
Grissel Ríos ◽  
Luis M. Vilá

Diffuse alveolar hemorrhage (DAH) is a life-threatening complication of systemic lupus erythematosus (SLE). Although infrequent, its mortality is very high. While there are no established therapeutic guidelines, DAH has been traditionally managed with high-dose intravenous (IV) corticosteroids, cyclophosphamide, and plasma exchange. The efficacy of alternative therapies such as rituximab has been described only in a few cases. Herein, we report a 25-year-old Hispanic man who presented with acute-onset SLE manifested by polyarthralgia, nephritis, seizures, pancytopenia, severe hypocomplementemia, and elevated anti-dsDNA antibodies. His disease course was complicated by DAH. His condition was refractory to high-dose intravenous (IV) methylprednisolone pulses, IV cyclophosphamide, and plasmapheresis. Given the lack of clinical response, he was started on IV rituximab 375 mg/m2 weekly for a total of four courses. He rapidly improved after the first two doses. Over the next seven months, he did not present recurrent pulmonary symptoms. Follow-up chest computed tomography did not show residual abnormalities. This case, together with other reports, suggests that rituximab is an effective therapeutic option for DAH in SLE.


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