Table 1 Characteristics of 104 patients with DAH and according to the presence of a vasculitis or a connective tissue disorder
From: Severe diffuse alveolar hemorrhage related to autoimmune disease: a multicenter study
All patients (n = 104) | Connective tissue disorder (n = 25) | Vasculitis (n = 79) | p | |
|---|---|---|---|---|
A. Baseline characteristics | ||||
Demographics | ||||
Age, years, median [IQR] | 56 [32–68] | 42 [27–55] | 61 [39–71] | 0.003 |
Male gender, n (%) | 56 (54%) | 7 (29%) | 48 (61%) | 0.02 |
Comorbidities | ||||
Charlson score, median [IQR] | 3 [1–4] | 2 [1–3.5] | 3 [1–4] | 0.23 |
Previous steroid treatment, n (%) | 26 (25%) | 10 (40%) | 16 (20%) | 0.064 |
Systemic disease,n(%) | ||||
Connective tissue disorder | 25 (24%) | NA | NA | |
Systemic lupus erythematosus | 12 (48%) | |||
Primary antiphospholipid syndrome | 9 (36%) | |||
Others* | 4 (16%) | |||
Vasculitis | 79 (76%) | NA | NA | |
ANCA-associated vasculitis | 57 (72%) | |||
Anti-GBM disease | 12 (15%) | |||
Cryoglobulin-associated vasculitis | 4 (5%) | |||
IgA-associated vasculitis | 6 (8%) | |||
Inaugural | 76 (73%) | 13 (52%) | 63 (80%) | 0.01 |
Relapse | 28 (27%) | 12 (48%) | 16 (20%) | |
Systemic disease manifestations, n (%) | ||||
DAH | 104 (100%) | 25 (100%) | 79 (100%) | 1 |
Hemoptysis | 53 (51%) | 10 (40%) | 43 (54%) | 0.25 |
Cough | 68 (65%) | 17 (68%) | 51 (65%) | 0.81 |
Acute respiratory failure | 78 (75%) | 20 (80%) | 58 (73%) | 0.6 |
Renal | 83 (81%) | 14 (56%) | 69 (89%) | 0.0009 |
Digestive | 7 (7%) | 2 (8%) | 5 (6%) | 0.68 |
Nervous system | 16 (16%) | 6 (24%) | 10 (13%) | 0.21 |
Joint | 20 (19%) | 4 (16%) | 16 (21%) | 0.78 |
Skin | 23 (22%) | 7 (28%) | 16 (21%) | 0.42 |
ICU admission characteristics, median [IQR] | ||||
SAPS II | 36 [25.75–47] | 33 [26–42] | 38 [26–48] | 0.51 |
Temperature | 37.9 [37–38.6] | 38.2 [37.2–39.1] | 37.7 [37–38.5] | 0.15 |
Oxygen flow, L/min | 15 [5–15] | 12 [5–15] | 15 [5–15] | 0.92 |
PaO2/FiO2 ratio | 150 [87–229] | 120 [90–230] | 157 [86–225] | 0.64 |
Time (days) from dyspnea onset to ICU admission | 5 [1–21] | 1 [0–10] | 6 [1–24] | 0.018 |
Time (days) from first symptoms to ICU admission | 30 [11.8–79] | 17 [10–46] | 32 [15–88] | 0.079 |
Time (days) from hospital admission to ICU admission | 5 [1–12] | 7 [3–15] | 3 [0–12] | 0.13 |
Biological findings at ICU admission, median [IQR] | ||||
Hemoglobin, g/L | 86 [72–95] | 87 [75–97] | 86 [72–95] | 0.73 |
Leukocytes, G/L | 11.7 [8.9–15.6] | 11.1 [6.4–17.1] | 11.7 [9.2–15.4] | 0.47 |
Platelets count, G/L | 257 [141–353] | 102 [53–236] | 278 [186–401] | < 0.0001 |
Creatinine, μmol/L | 235 [92–433] | 103 [71–174] | 303 [128–520] | 0.0002 |
Urine protein/creatinine, g/mmol | 0.19 [0.1–0.33] | 0.12 [0.07–0.25] | 0.2 [0.1–0.34] | 0.17 |
LDH, mmol/L | 488 [354–905] | 873 [532–1152] | 454 [329–714] | 0.008 |
B. ICU management. | ||||
ICU management,n(%) | ||||
Vasopressor use | 36 (35%) | 8 (32%) | 28 (35%) | 0.81 |
Renal replacement therapy | 55 (53%) | 6 (24%) | 49 (62%) | 0.001 |
Respiratory management | ||||
Non-invasive ventilation | 33 (32%) | 8 (32%) | 25 (32%) | 1 |
Mechanical ventilation | 52 (50%) | 11 (44%) | 41 (52%) | 0.65 |
ARDS diagnosis | 52 (50%) | 13 (52%) | 43 (55%) | 0.82 |
Prone positioning | 11 (21%) | 3 (12%) | 8 (11%) | 1 |
Mechanical ventilation duration, days, median [IQR] | 12 [6–22] | 4 [4–18] | 13 [9–33] | 0.007 |
Red blood cell transfusion | 78 (75%) | 16 (64%) | 62 (80%) | 0.33 |
Systemic disease management,n(%) | ||||
Time (days) from first symptoms to diagnosis | 31 [11.5–77] | 19.5 [10–53] | 32 [15–86] | 0.14 |
Steroids | 103 (99%) | 25 (100%) | 78 (99%) | 1 |
Steroids pulse therapy | 93 (89%) | 20 (80%) | 73 (92%) | 0.13 |
Cyclophosphamide | 72 (69%) | 9 (36%) | 63 (80%) | < 0.0001 |
Rituximab | 12 (11.5%) | 1 (4%) | 11 (14%) | 0.29 |
PLEX | 57 (55%) | 6 (24%) | 51 (65%) | 0.0009 |
Number of PLEX | 7 [4.3–9.5] | 4 [2–9] | 7 [5–9] | 0.32 |
Bacterial superinfection,n(%) | 30 (29%) | 3 (12%) | 27 (34%) | 0.042 |
Hospital mortality,n(%) | 16 (15%) | 3 (12%) | 13 (17%) | 0.76 |
Follow-up | 48 (55%) | 13 (59%) | 35 (53%) | 0.65 |
Length of follow-up (months) | 19 [8–38] | 37 [11–66] | 15 [7–28] | 0.003 |
Chronic renal failure** | 22 (65%) | 4 (80%) | 18 (62%) | 0.33 |
Dialysis** | 8 (24%) | 1 (20%) | 7 (24%) | 0.42 |