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Systematic Review of Diffuse Alveolar Hemorrhage in Systemic Lupus Erythematosus Focus on Outcome and Therapy

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Systematic Review of Diffuse Alveolar Hemorrhage in Systemic Lupus Erythematosus Focus on Outcome and Therapy REVIEW Systematic Review of Diffuse Alveolar Hemorrhage in Systemic Lupus Erythematosus Focus on Outcome and Therapy Christina Ednalino, MD, Julie Yip, DO, and Steven E. Carsons, MD to be an adjuvant therapy in SLE patients not only for DAH, but Background: Diffuse alveolar hemorrhage (DAH) is an uncommon but other severe manifestations such as glomerulonephritis and potentially life-threatening manifestation of systemic lupus erythematosus neuropsychiatric SLE. Because of the rarity of DAH in SLE, pro- (SLE) associated with high mortality. Although survival and its associated spective studies are difficult to perform, and most of the published clinical, laboratory, and therapeutic features have been reported for case re- literature on this condition is in the form of case reports and case se- ports and series, they have not been systematically reviewed. ries. We have conducted a systematic review of 140 subjects with Objectives: The purpose of this systematic review was to assess survival DAH in SLE, to evaluate survival with adjunctive plasmapheresis. of episodes of DAH in SLE over 3 decades and to categorize trends in ther- apies, commonly utilized to treat this disorder. Results: Overall, SLE patients survived 61% of 174 DAH episodes METHODS representing 140 patients. Episode survival was 67% in the time period A PubMed search was performed using the terms [Alveolar from 2000 to 2013. Corticosteroids were nearly universally used therapeu- OR Pulmonary Hemorrhage] AND [Systemic Lupus Erythemato- tically, and cyclophosphamide was used in 55%. Plasmapheresis was used sus] for all publications between 1981 and February 2014. Case in 31% and did not appear to be associated with survival. reports and series where patients had a diagnosis of SLE in addi- Conclusions: Diffuse alveolar hemorrhage in SLE still carries a high tion to diffuse alveolar hemorrhage (DAH) were included. Exclu- risk of mortality; however, survival trends appear to demonstrate an in- sion criteria included age younger than 18 years, pregnancy, crease from approximately 25% in the 1980s to 67% in the current decade. antineutrophil cytoplasmic antibody (ANCA) positivity, and other Increased use of cyclophosphamide appears to be associated with better conditions known to cause pulmonary hemorrhage such as con- survival, whereas plasmapheresis does not appear to influence outcome. comitant antiphospholipid antibody syndrome, uremia, dissemi- Although these results need to be interpreted with caution because they nated intravascular coagulation, or cytomegalovirus pneumonia. are not derived from randomized controlled trials, we believe this repre- We also excluded articles in which individual survival data were sents the largest reported compilation of survival data in DAH associated not available. The 2 searches yielded 395 results. The search re- with SLE. sults were cross-referenced for duplicates. Abstracts were ex- cluded if any of the exclusion criteria were apparent, or if the Key Words: diffuse alveolar hemorrhage, plasmapheresis, article was not in English. Fifty-nine full articles were reviewed systemic lupus erythematosus to ensure that inclusion and exclusion criteria were satisfied. A (J Clin Rheumatol 2015;21: 305–310) total of 44 articles were selected for final analysis. There were 24 single case articles and 20 multiple case articles. The largest iffuse alveolar hemorrhage (DAH) is an uncommon, poten- number of analyzed cases in a single article was 22, and only D tially life-threatening manifestation of systemic lupus erythe- 2 articles contained greater than 10 analyzed cases. Qualifying matosus (SLE), occurring in less than 2% of patients.1–4 Diffuse publications were reviewed, and data entered into a predesigned alveolar hemorrhage in SLE has historically been associated with electronic spreadsheet. Clinical manifestations, laboratory and ra- a high mortality; the majority of reported rates falling between diographic data, treatment, and survival outcome were analyzed 70% and 90%.1,4–6,9 The condition is typically characterized by for cases where such specific information was given. The utilization shortness of breath with or without hemoptysis, new infiltrates of plasmapheresis was examined by subject, as well as by episode in on chest radiography, and a drop of hemoglobin of at least the event multiple episodes of DAH occurred for a single subject. 1.5 g/dL. While the pathogenesis of DAH is still not well under- Selected treatment and outcome variables were further analyzed stood, reported histological findings include bland alveolar hemor- by decade. Association of survival with treatment was analyzed rhage without vasculitis, diffuse alveolar damage, and interstitial using Fisher exact test (Graphpad Software Inc., La Jolla, CA). inflammation.1,10,11 Current management for DAH in SLE includes high-dose intravenous corticosteroids, intravenous cyclophospha- RESULTS 12 mide, and extensive supportive care. Plasmapheresis (PF) has been Twenty-seven articles comprising 80 cases were published be- used in SLE patients since the early 1980s; however, the earliest 13 tween 2000 and 2013, 9 articles comprising 48 subjects were pub- studies failed to confirm their benefit. Nevertheless, PF continues lished between 1990 and 1999, and 8 articles with 19 total subjects were from 1980 to 1989. Publication dates did not always corre- ’ From the Division of Rheumatology, Allergy & Immunology, Winthrop- spond with the decade in which a patient s episode occurred. There University Hospital, Clinical Campus of Stony Brook University School were 140 patients with 174 episodes of DAH among them. Ages of Medicine, Mineola, New York. ranged from 18 to 72 years. Eighty-five percent were female. S.E.C. has consulted with Biogen-Idec. The other authors declare no conflict of interest. Correspondence: Steven E. Carsons, MD, Division of Rheumatology, Allergy & Clinical Manifestations Of SLE In Patients With DAH Immunology, Winthrop-University Hospital, 120 Mineola Blvd, Suite 410, In patients for whom individual data were available, clini- Mineola, NY 11501. E-mail: [email protected]. Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. cal manifestations were analyzed (Table 1). Forty-four percent ISSN: 1076-1608 had joint involvement, 60% had mucocutaneous symptoms, and DOI: 10.1097/RHU.0000000000000291 25% had neuropsychiatric involvement. Seventy-three percent JCR: Journal of Clinical Rheumatology • Volume 21, Number 6, September 2015 www.jclinrheum.com 305 Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. Ednalino et al JCR: Journal of Clinical Rheumatology • Volume 21, Number 6, September 2015 Association Of Survival With Therapeutic TABLE 1. Patient Characteristics of the 140 Cases Reviewed Modalities Age (range), y 18–72 Overall, patients survived 107 (61%) of 174 episodes; 73 Female-male ratio 85:15 (52%) of 140 cases survived. Of those who received plasmaphere- sis, 23 (53%) of 43 survived (Table 3). In the cases where PF was Nephritis 73% not used, survival was 50 (51%) of 97. When analyzed by epi- Other renal 9% sodes, 65% (35/54) were survived with plasmapheresis. If PF Cytopenias 71% was not used, episode survival was 60% (72/120) (not statistically Mucocutaneous 25% significant). Seventy (71%) of 98 episodes where cyclophospha- Articular 44% mide was used resulted in survival. Patients survived 37 (49%) Dyspnea on presentation 90% of 76 episodes where cyclophosphamide was not used. (relative Hemoptysis 57% risk, 0.68; P = 0.0028). In patients receiving cyclophosphamide, Elevated dsDNA antibody 75% data on pheresis use were available for 71 episodes; pheresis was Low complement 86% used in 24 (33.8%). For patients not receiving cyclophosphamide, data on pheresis use were available for 68 episodes; pheresis was used in 13 (19.1%). Thus, pheresis was used more often in pa- tients who were treated with cyclophosphamide. had prior or active glomerulonephritis, and an additional 9% had renal abnormalities ranging from elevated creatinine or urinary protein, abnormal urinary sediment, or unspecified kidney in- Infection And Outcomes volvement. Upon presentation 89 (90%) of 92 had dyspnea, and The cause of death was provided for 36 cases. Forty-four 71 (57%) of 124 patients had hemoptysis. One hundred two epi- percent were from pulmonary hemorrhage alone, 28% were sodes presenting with hemoptysis had outcome data reported. listed as either cardiopulmonary or pulmonary failure. In 8 Sixty episodes presented with hemoptysis, and 39 (65%) sur- (22%), infection was listed as a cause of death. In 5 cases, in- vived. Forty-two episodes presented without hemoptysis and 26 fection was primary (1 each: pneumonia, left lower lobe pneu- (62%) survived. monia, Acinetobacter sepsis, pneumocystis pneumonia, and Pseudomonas sepsis), and in 3, it was contributory (hemor- rhagic pneumonitis with bronchopneumonia, Legionella sepsis Laboratory Data And Clinical Outcome with pulmonary hemorrhage, and pulmonary hemorrhage Seventy (61%) of 114 had cytopenias, 62 (75%) of 83 had with infection). elevated double-stranded DNA, and 84 (86%) of 98 had hypo- The use of antibiotics was reported for 82 episodes. Antibi- complementemia. Despite the high prevalence of hypocomplemen- otics were used in 60; 41 survived (68%). Antibiotics were not temia overall, the normocomplementemic patients (24 episodes) used in 22; 14 survived (64%). had a lower survival (8 episodes; 33%) as compared with the hypocomplementemic patients (99 episodes), where 81 episodes
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