scholarly journals AB0366 DIFFUSE ALVEOLAR HEMORRHAGE IN LUPUS NEPHRITIS PATIENTS: MULTICENTER RECTROSPECTIVE STUDY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1483.1-1484
Author(s):  
S. Abdulaziz ◽  
H. Halabi ◽  
S. Bahlas ◽  
S. Attar ◽  
M. Dessougi ◽  
...  
Keyword(s):  
Risk Factors ◽  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Clinical Presentation ◽  
Pulmonary Hemorrhage ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Systemic Lupus ◽  
Cns Involvement

Background:Diffuse alveolar hemorrhage (DAH) is a rare and potentially lethal complication of systemic lupus erythematosus (SLE) with a high mortality rate. It occurs more frequently in patients with lupus nephritis (LN).Objectives:The aim of our study is to explore the characteristics of patients that develop DAH with lupus nephritis, risk factors that predispose DAH, treatment response and outcomes.Methods:Multicenter retrospective cohort study was undertaken including 6 centers in Saudi Arabia from 2002 to 2018. Systemic lupus erythematosus patients meeting the SLICC criteria with lupus nephritis (biopsy proven or proteinuria or renal impairment due to lupus) presenting with diffuse alveolar hemorrhage (fulfilling a predefined criteria) were included in the study. An identical number of control group with lupus nephritis was also studied. Data was obtained from medical records by using a data sheet: demographics including age, gender, diagnosis, date of diagnosis of lupus, date of presentation of alveolar hemorrhage, clinical presentation, detection of alveolar hemorrhage proved by radiology, lavage or biopsy and laboratory parameters: including level of hemoglobin before and during DAH, sign of activity, treatment and outcome of DAH. Identification of risk factors predisposing to DAH in lupus nephritis patients was analyzed.Results:We identified 23 cases of DAH with lupus nephritis, all fulfilling the criteria. Mean age at presentation of DAH was 31.09 ± 12.6 years ranging from 14-57 years, of which 87 % were females. 13 patients 56.5% had Class 4 LN and 21.7% had Class 4 and 5 LN on renal pathology. DAH occurred at a mean of 6.5 years ±3.8 in 13/23 patients with LN. Shortness of breath 95%, new chest x ray finding 95.7% and mean drop of haemoglobin of 2.72 gm/dl ±0.97 were more frequent at presentation of DAH with LN patients. High SLE disease activity - SELENA SLEDAI 2K was 38.56 ±19.3 was present at the onset of DAH. All were treated with methyprednisone,15/23 (65.2%) underwent mechanical ventilation and plasmapheresis was done in 21/23 patients (91.3%). Cyclophosphamide was given in 14/21 patients (60.9%), Intravenous immunoglobulins were given in 14/23 patients (65.2%) and dialysis was done in 12/23 patients (52.2%). Mortality occurred 8 patients 34.8 %. In comparison with the LN group, a mean haemoglobin of 7.56 ± 1.3, CNS involvement, vasculitis and fever>38% were of statistically significance P value: <0.001,0.02,0.03 and 0.03 respectively.Conclusion:In this multicenter cohort series with DAH in LN patients CNS involvement, vasculitis and fever>38 were associated in the occurrence of DAH. Mortality was low in our cohort in comparison to previous series which may be explained by early diagnosis and use of aggressive management.Well designed prospective studies are required to identify high risk patients for preventing this serious complication.References:[1]Eagen JW, Memoli VA, Roberts JL, et al. Pulmonary hemorrhage in systemic lupus erythematosus. Medicine (Baltimore) 1978; 57:545.[2]Badsha H, Teh CL, Kong KO, et al. Pulmonary hemorrhage in systemic lupus erythematosus. Semin Arthritis Rheum 2004; 33:414.[3]Zamora MR, Warner ML, Tuder R, Schwarz MI. Diffuse alveolar hemorrhage and systemic lupus erythematosus. Clinical presentation, histology, survival, and outcome. Medicine (Baltimore) 1997; 76:192.[4]Hsu BY, Edwards DK 3rd, Trambert MA. Pulmonary hemorrhage complicating systemic lupus erythematosus: role of MR imaging in diagnosis. AJR Am J Roentgenol 1992; 158:519.Disclosure of Interests:None declared

scholarly journals An uncommon cause of diffuse alveolar hemorrhage in a young male

2019 ◽  
Vol 89 (2) ◽  
Author(s):  
Anshul Mittal ◽  
Jagdish Chander Suri ◽  
Shibdas Chakrabarti ◽  
Pranav Ish
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Renal Involvement ◽  
Young Male ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Systemic Lupus ◽  
Threatening Condition ◽  
Life Threatening

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.

scholarly journals Diffuse Alveolar Hemorrhage in Systemic Lupus Erythematosus: Histopathologic Features and Clinical Correlations

2017 ◽  
Vol 2017 ◽  
pp. 1-6 ◽  
Author(s):  
Robert Ta ◽  
Romulo Celli ◽  
A. Brian West
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Diffuse Alveolar Damage ◽  
Acute Infection ◽  
Pulmonary Hemorrhage ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Medical Emergency ◽  
Systemic Lupus ◽  
American Girl

The case of a 16-year-old African-American girl with systemic lupus erythematosus, who developed diffuse alveolar hemorrhage with fatal consequences, is described. Diffuse alveolar hemorrhage is a rare but serious complication of systemic lupus. It occurs in three distinct but overlapping phenotypes, acute capillaritis, bland pulmonary hemorrhage, and diffuse alveolar damage, each of which is associated with a different group of underlying conditions. Diffuse alveolar hemorrhage is a medical emergency: choice of treatment depends on early diagnosis and determination of the underlying etiology. Acute infection, superimposed on diffuse alveolar hemorrhage in the setting of immune compromise, is often a terminal event, as in this case.

scholarly journals A Fatal Case of Diffuse Alveolar Hemorrhage in the Setting of Systemic Lupus Erythematosus: A Case Report and Review of Noninfectious Causes of Acute Pulmonary Hemorrhage in Adults

2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Mia C. Lundgren ◽  
Jerry A. Molitor ◽  
Benjamin Spilseth ◽  
Oyedele Adeyi
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Systemic Vasculitis ◽  
Fatal Case ◽  
Pulmonary Hemorrhage ◽  
Disease Process ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Systemic Lupus ◽  
Autoantibody Production

Systemic lupus erythematosus (SLE) is an autoimmune connective tissue disease, characterized by autoantibody production and immune complex formation, that has the potential to affect virtually any organ. Pleuropulmonary involvement occurs in 50–70% and commonly manifests as pleuritis and pleural effusion. Diffuse alveolar hemorrhage (DAH) is a rare manifestation of SLE. Most cases of DAH occur in young adults with an underlying autoimmune disease such as systemic vasculitis or Goodpasture syndrome. SLE is typically lower on the list of initial differential diagnoses of DAH due to its rarity compared to other etiologies. We present a case of a patient with dyspnea on exertion, dry coughs, lower extremity edema, and intermittent periorbital edema who ultimately succumbed to respiratory failure secondary to DAH in the setting of SLE. The diagnosis of SLE was suspected clinically and confirmed at autopsy due to her rapid clinical deterioration. DAH requires prompt intervention, and management is guided by the underlying disease process. SLE is a potentially treatable disease; therefore, timely diagnosis is important in order to exclude other noninfectious causes of DAH (reviewed in this report) and to initiate appropriate therapy.

Systemic lupus erythematosus–associated diffuse alveolar hemorrhage: a single-center, matched case–control study in China

Lupus ◽  
2020 ◽  
Vol 29 (7) ◽  
pp. 795-803 ◽  
Author(s):  
Yiduo Sun ◽  
Cong Zhou ◽  
Jiuliang Zhao ◽  
Qian Wang ◽  
Dong Xu ◽  
...  
Keyword(s):  
Risk Factors ◽  
Systemic Lupus Erythematosus ◽  
Multivariate Analysis ◽  
Prognostic Factors ◽  
Lupus Erythematosus ◽  
Case Control ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Single Center ◽  
Systemic Lupus

Background This study described clinical characteristics and outcome in systemic lupus erythematosus (SLE) patients with diffuse alveolar hemorrhage (DAH), and investigated risk factors and prognostic factors for DAH. Methods We conducted a retrospective nested case–control analysis in a single-center cohort. We enrolled 94 SLE patients with DAH. For each case of DAH, two age-, sex-, and SLE courses–matched controls were randomly selected from our cohort. All patients were enrolled between 2004 and 2019 and were followed until death, end of registration with the physician’s practice, or end of January 2019. We estimated the risk factors for DAH and prognostic factors for mortality using multivariate analysis. Results We included 4744 patients diagnosed with SLE, with 94 cases of DAH, for an incidence rate of 2.0%. DAH may occur in any stage of SLE but mostly in the early phase of disease course. Lupus nephritis (LN) was the most common concomitant involvement at DAH diagnosis. By multivariate analysis, LN, anti-SSA positivity, thrombocytopenia and elevated C-reactive protein (CRP) were significantly associated with DAH in SLE patients. All-cause mortality was increased in SLE with DAH compared with SLE without DAH (adjusted hazard ratio 6.0, 95% confidence interval 2.8–13.0, p < 0.0001). Intravenous cyclophosphamide (CTX) showed an increased tendency for better survival in DAH after adjusting for Systemic Lupus Erythematosus Disease Activity Index 2000, acute kidney injury and mechanical ventilation. Conclusions LN, anti-SSA positivity, thrombocytopenia and elevated CRP were independent risk factors of DAH in lupus patients. Due to a high early death rate of DAH and little long-term damage, DAH patients may benefit from early diagnosis and intensive treatment, and CTX-based therapy can be a preferential choice.

scholarly journals Strongyloidiasis and Diffuse Alveolar Hemorrhage in a Patient with Systemic Lupus Erythematosus

2014 ◽  
Vol 2014 ◽  
pp. 1-3 ◽  
Author(s):  
Fernando Gonzalez-Ibarra ◽  
Parag Chevli ◽  
Lindsey Schachter ◽  
Maninder Kaur ◽  
Sahar Eivaz-Mohammadi ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Immunosuppressive Therapy ◽  
Lupus Erythematosus ◽  
Clinical Status ◽  
Strongyloides Stercoralis ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Systemic Lupus ◽  
Recent Diagnosis

The presence ofStrongyloides stercoralisinfection in patients with systemic lupus erythematosus (SLE) has been described previously.Strongyloides stercoralishyperinfection syndrome (SHS) that usually develops in patients under immunosuppressive therapy may affect a variety of organs, but the presentation with diffuse alveolar hemorrhage (DAH) is rare with only a few cases described in the literature. We present the case of a 36-year-old Hispanic female with a past medical history relevant for SLE and a recent diagnosis of lupus nephritis and hypertension. The patient who developed sudden and progressive abdominal pain and respiratory distress, with the presence of bilateral crackles and severe hypoxemia, is currently under treatment with steroids and cyclophosphamide for worsening of lupus nephritis. The patient underwent endotracheal intubation and mechanical ventilation, and computed tomography showed the presence of bilateral pulmonary infiltrates suggestive of DAH. Bronchoalveolar lavage was done and showed the presence of filariform larvae, morphologically consistent withStrongyloides stercoralis. Treatment with ivermectin was started and patient responded to treatment with improvement of clinical status. In conclusion, the development of SHS in patients with lupus, especially when receiving immunosuppressive therapy, is a severe and potentially fatal complication. Early detection and treatment may decrease mortality.

Risk of osteonecrosis in systemic lupus erythematosus: An 11-year Chinese single-center cohort study

Lupus ◽  
2021 ◽  
pp. 096120332110211
Author(s):  
Yin Long ◽  
Shangzhu Zhang ◽  
Jiuliang Zhao ◽  
Hanxiao You ◽  
Li Zhang ◽  
...  
Keyword(s):  
Risk Factors ◽  
Systemic Lupus Erythematosus ◽  
Risk Factor ◽  
Femoral Head ◽  
Lupus Erythematosus ◽  
Femoral Head Necrosis ◽  
Joint Involvement ◽  
Multiple Site ◽  
Systemic Lupus ◽  
Cns Involvement

Objective Osteonecrosis (ON), which can lead to physical disability, is a common complication of systemic lupus erythematosus (SLE). The purpose of this study was to determine the prevalence of ON and identify possible risk factors in Chinese SLE patients. Methods SLE patients who fulfilled the 1997 American College of Rheumatology SLE classification criteria were recruited from the Peking Union Medical College Hospital. The chi-square test (χ 2 test) and multivariate regression analyses were used to evaluate risk factors. The Cox proportional-hazards model was used to construct the survival curves and estimate the simultaneous effects of prognostic factors on survival. Results We consecutively enrolled 1,158 patients, of which 88 patients (7.6%) developed ON. Among ON patients, 57.1% of patients had isolated femoral head necrosis and 42.9% had multiple joint involvement. The mean age of ON patients (24.62 ± 8.89 years) was significantly younger than SLE patients without ON (27.23 ± 10.16 years, p = 0.09). The ON group presented with a much longer disease course (10.68 ± 5.97 years, p < 0.001) and increased incidence of arthritis, kidney, and central nervous system (CNS) involvement (65.9% [ p < 0.05], 57.6% [ p < 0.05], and 16.5% [ p < 0.05], respectively, in the ON group). ON patients were more likely to be treated with glucocorticoid (GC) and to receive a high dose of prednisolone at the initial stage of SLE ( p < 0.05). The percentage of patients who received hydroxychloroquine was much higher in the control group ( p < 0.001). Cox regression analysis suggested that CNS involvement and GC therapy were two independent risk factors for ON in SLE patients. The presence of anti-phospholipid antibodies (aPLs) was a risk factor for multiple joint necrosis (odds ratio: 6.28, p = 0.009). Conclusions ON remains a serious and irreversible complication in SLE. In addition to glucocorticoid therapy, we found that CNS system involvement was a risk factor for ON, while the administration of hydroxychloroquine was a protective factor. The clinical characteristics of multiple site ON patients were distinct from isolated femoral head necrosis patients. The presence of aPLs was a risk factor for multiple site osteonecrosis.

Sign in / Sign up

Export Citation Format

Share Document