title: Alveolar hemorrhage: distinct features of juvenile and adult onset systemic lupus erythematosus.
abstract: We compared outcomes of alveolar hemorrhage (AH) in juvenile (JSLE) and adult onset SLE (ASLE). From 263 JSLE and 1522 ASLE, the AH occurred in 13 (4.9%) and 15 (1.0%) patients, respectively (p < .001). Both groups had comparable disease duration (2.6 ± 3.0 vs. 5.6 ± 7.0 years, p = .151) and median SLEDAI scores [17.5 (2 to 32) vs. 17.5 (3 to 28), p = 1.000]. At AH onset, a higher frequency of JSLE were already on a high prednisone dose ( > 0.5 mg/kg/day) compared to ASLE (54% vs. 15%, p = .042). The mean drop of hemoglobin was significantly lower in JSLE (2.9 ± 0.9 vs. 5.5 ± 2.9 g/dL, p = .006). Although treatments with methylprednisolone, plasmapheresis, intravenous immunoglobulin and cyclophosphamide were similar in both groups (p > .050), regarding outcomes, there was a trend in high frequency of mechanical ventilation use (85% vs. 47%, p = .055) and also significant mortality (69% vs. 13%, p = .006) in JSLE compared to ASLE. The sepsis frequency was comparable in both groups (50% vs. 27%, p = .433). We have identified that AH in JSLE has a worse outcome most likely related to respiratory failure. The AH onset in JSLE already treated with high-dose steroids raises the concern of inadequate response to this treatment and reinforces the recommendation of early aggressive alternative therapies in this group of patients.
authors.affiliation: Rheumatology Division, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, Brazil.
authors.forename: D B
authors.initials: DB
authors.lastname: Araujo
authors.forename: E F
authors.initials: EF
authors.lastname: Borba
authors.forename: C A
authors.initials: CA
authors.lastname: Silva
authors.forename: L M A
authors.initials: LM
authors.lastname: Campos
authors.forename: R M R
authors.initials: RM
authors.lastname: Pereira
authors.forename: E
authors.initials: E
authors.lastname: Bonfa
authors.forename: S K
authors.initials: SK
authors.lastname: Shinjo
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D002648:Child; D004417:Dyspnea / Q000209:etiology; D005260:Female; D006454:Hemoglobins / Q000378:metabolism; D006469:Hemoptysis / Q000209:etiology; D006470:Hemorrhage / Q000097:blood / Q000188:drug therapy / Q000209:etiology*; D006801:Humans; D000860:Hypoxia / Q000209:etiology; D008171:Lung Diseases / Q000097:blood / Q000188:drug therapy / Q000209:etiology*; D008180:Lupus Erythematosus, Systemic / Q000097:blood / Q000150:complications / Q000188:drug therapy* / Q000401:mortality*; D055501:Macrophage Activation Syndrome / Q000209:etiology; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use; D011650:Pulmonary Alveoli; D012121:Respiration, Artificial; D012189:Retrospective Studies; D018805:Sepsis / Q000209:etiology; D018709:Statistics, Nonparametric; D055815:Young Adult
chemical_list: D000893:Anti-Inflammatory Agents; D006454:Hemoglobins; D011241:Prednisone; D008775:Methylprednisolone