Demographic, clinical and laboratory manifestations of study population at onset
| Demographic characteristics | |
| Female/Male | 104/135 |
| Ethnicity, n (%) | |
| Caucasian ancestry | 190 (79.5) |
| African | 26 (10.9) |
| Asian | 17 (7.1) |
| Latin-American | 6 (2.5) |
| Age at onset, median (IQR), years | 8 (4–11) |
| Clinical manifestations | |
| At least one skin involvement, n (%) | 155 (64.9) |
| Maculopapular rash | 106 (44.4) |
| Multiform erythema | 23 (9.6) |
| Perineal erythema | 3 (1.3) |
| Scarlattinoform rash | 8 (3.4) |
| Palmoplantar erythema | 41 (17.2) |
| At least one mucosal involvement, n (%) | 154 (64.4) |
| Cheilitis | 97 (40.6) |
| Oral changes | 90 (37.7) |
| At least one ocular involvement, n (%) | 135 (56.5) |
| Conjunctivitis | 134 (56.1) |
| At least one neurological involvement, n (%) | 112 (46.9) |
| Headache | 42 (17.6) |
| Aseptic meningitis | 11 (4.6) |
| Irritability | 56 (23.4) |
| At least one gastrointestinal involvement, n (%) | 174 (72.8) |
| Diarrhoea | 105 (43.9) |
| Vomiting | 73 (30.6) |
| Abdominal pain | 112 (46.9) |
| Colecyts hydrops | 6 (2.5) |
| Acute pancreatitis | 3 (1.3) |
| Hepatomegaly | 30 (12.6) |
| Splenomegaly | 21 (8.8) |
| At least one lung involvement, n (%) | 80 (33.5) |
| Dyspnea | 27 (11.3) |
| Tachypnea | 28 (11.7) |
| Lobar pneumonia | 12 (5.0) |
| Interstitial pneumonia | 22 (9.2) |
| Oxygen supplementation | 37 (15.5) |
| Non-invasive/Invasive ventilation | 21 (8.8) |
| At least one heart involvement, n (%) | 169 (70.7) |
| Myocarditis | 64 (26.8) |
| Pericarditis | 32 (13.4) |
| Valvular insufficiency | 46 (19.3) |
| Coronary dilation | 22 (9.2) |
| Coronary aneurism | 8 (3.4) |
| Heart failure | 39 (16.4) |
| Hypotension/Non-cardiogenic shock | 46 (19.3) |
| Cardiac arrest | 2 (0.8) |
| At least one osteoarticular involvement, n (%) | 30 (12.6) |
| Arthritis/arthralgia | 19 (8.0) |
| Myositis | 4 (1.7) |
| Lymphadenopathy, n (%) | 72 (30.1) |
| Laboratory exams (normal values) | Median (IQR) |
| Leucocytes (5-19.5 103/mmc) | 10 795 (7750-13 615) |
| Neutrophils (1-9 103/mmc) | 8280 (5650-10450) |
| Lymphocytes (2.5-16.5 103/mmc) | 1070 (700-1950) |
| Monocytes (0.2-1.10 103/mmc) | 400 (220-780) |
| Haemoglobin (9-13.5 g/dL) | 11.3 (10.4-12.3) |
| Platelets (100-400/mmc) | 203 000 (121 000-275 000) |
| ALT (10-50 U/L) | 26 (18-44) |
| AST (28-41 U/L) | 34 (23-50) |
| GammaGT (39-308 U/L) | 23 (15-48) |
| Creatinine (0.17-0.43 mg/dL) | 0.5 (0.4-0.8) |
| Sodium (136-145mEq/L) | 134 (131-136) |
| Clorus (98-107mEq/L) | 99 (96-102) |
| Potassium (3.4-4.5mEq/L) | 4 (3.5-4.3) |
| CRP (<5mg/L) | 146.5 (97.0-227.1) |
| IgA (*mg/dL) | 102 (77-173) |
| IgG (*mg/dL) | 731.5 (591.5-993.5) |
| IgM (*mg/dL) | 98.5 (74-136.5) |
| Proteins (5.9-7.3 g/dL) | 6.3 (5.5-6.9) |
| Albumin (3.1-5.2 g/dL) | 3.1 (2.7-3.6) |
| CPK (39-308 U/L) | 66.5 (41-148) |
| pH (7.32–7.42) | 7.42 (7.38–7.46) |
| pCO2 (41–51mmHg) | 35 (31–39) |
| Bicarbonates (22–29 mmol/l) | 22.9 (21.1–25.4) |
| Lactic acid (<3.1 mmol/l) | 1.4 (1.2–1.9) |
| Troponin I (<40 ng/l) | 84.7 (26–398) |
| Troponin T (14 ng/l) | 24.6 (8–62.5) |
| Hs-troponin (<11 ng/l) | 33 (11.7–110.1) |
| Triglycerides (<150 mg/dl) | 184 (131–251) |
| ESR (<28 mm/h) | 53.5 (35–74) |
| Cholesterol (<190 mg/dl) | 111 (86–129) |
| Fibrinogen (170–420 mg/dl) | 582 (468–717) |
| INR | 1.2 (1.1–1.4) |
| aPTT (0.8–1.20 ratio) | 1.2 (1.1–1.3) |
| ATIII (79–120%) | 81 (73–93) |
| C3 (90-180 mg/dl) | 134 (89–153) |
| C4 (10-40 mg/dl) | 23.5 (13–32) |
| Ferritin (30-400 ng/ml) | 540 (287.5–915.5) |
| D-dimer (<500 ng/ml) | 2222 (1185.5–3821.5) |
| NT-proBNP (<400 pg/ml) | 3364.5 (873.9–9222.5) |
| BNP (<100 pg/ml) | 557.5 (135–1110) |
| Demographic characteristics | |
| Female/Male | 104/135 |
| Ethnicity, n (%) | |
| Caucasian ancestry | 190 (79.5) |
| African | 26 (10.9) |
| Asian | 17 (7.1) |
| Latin-American | 6 (2.5) |
| Age at onset, median (IQR), years | 8 (4–11) |
| Clinical manifestations | |
| At least one skin involvement, n (%) | 155 (64.9) |
| Maculopapular rash | 106 (44.4) |
| Multiform erythema | 23 (9.6) |
| Perineal erythema | 3 (1.3) |
| Scarlattinoform rash | 8 (3.4) |
| Palmoplantar erythema | 41 (17.2) |
| At least one mucosal involvement, n (%) | 154 (64.4) |
| Cheilitis | 97 (40.6) |
| Oral changes | 90 (37.7) |
| At least one ocular involvement, n (%) | 135 (56.5) |
| Conjunctivitis | 134 (56.1) |
| At least one neurological involvement, n (%) | 112 (46.9) |
| Headache | 42 (17.6) |
| Aseptic meningitis | 11 (4.6) |
| Irritability | 56 (23.4) |
| At least one gastrointestinal involvement, n (%) | 174 (72.8) |
| Diarrhoea | 105 (43.9) |
| Vomiting | 73 (30.6) |
| Abdominal pain | 112 (46.9) |
| Colecyts hydrops | 6 (2.5) |
| Acute pancreatitis | 3 (1.3) |
| Hepatomegaly | 30 (12.6) |
| Splenomegaly | 21 (8.8) |
| At least one lung involvement, n (%) | 80 (33.5) |
| Dyspnea | 27 (11.3) |
| Tachypnea | 28 (11.7) |
| Lobar pneumonia | 12 (5.0) |
| Interstitial pneumonia | 22 (9.2) |
| Oxygen supplementation | 37 (15.5) |
| Non-invasive/Invasive ventilation | 21 (8.8) |
| At least one heart involvement, n (%) | 169 (70.7) |
| Myocarditis | 64 (26.8) |
| Pericarditis | 32 (13.4) |
| Valvular insufficiency | 46 (19.3) |
| Coronary dilation | 22 (9.2) |
| Coronary aneurism | 8 (3.4) |
| Heart failure | 39 (16.4) |
| Hypotension/Non-cardiogenic shock | 46 (19.3) |
| Cardiac arrest | 2 (0.8) |
| At least one osteoarticular involvement, n (%) | 30 (12.6) |
| Arthritis/arthralgia | 19 (8.0) |
| Myositis | 4 (1.7) |
| Lymphadenopathy, n (%) | 72 (30.1) |
| Laboratory exams (normal values) | Median (IQR) |
| Leucocytes (5-19.5 103/mmc) | 10 795 (7750-13 615) |
| Neutrophils (1-9 103/mmc) | 8280 (5650-10450) |
| Lymphocytes (2.5-16.5 103/mmc) | 1070 (700-1950) |
| Monocytes (0.2-1.10 103/mmc) | 400 (220-780) |
| Haemoglobin (9-13.5 g/dL) | 11.3 (10.4-12.3) |
| Platelets (100-400/mmc) | 203 000 (121 000-275 000) |
| ALT (10-50 U/L) | 26 (18-44) |
| AST (28-41 U/L) | 34 (23-50) |
| GammaGT (39-308 U/L) | 23 (15-48) |
| Creatinine (0.17-0.43 mg/dL) | 0.5 (0.4-0.8) |
| Sodium (136-145mEq/L) | 134 (131-136) |
| Clorus (98-107mEq/L) | 99 (96-102) |
| Potassium (3.4-4.5mEq/L) | 4 (3.5-4.3) |
| CRP (<5mg/L) | 146.5 (97.0-227.1) |
| IgA (*mg/dL) | 102 (77-173) |
| IgG (*mg/dL) | 731.5 (591.5-993.5) |
| IgM (*mg/dL) | 98.5 (74-136.5) |
| Proteins (5.9-7.3 g/dL) | 6.3 (5.5-6.9) |
| Albumin (3.1-5.2 g/dL) | 3.1 (2.7-3.6) |
| CPK (39-308 U/L) | 66.5 (41-148) |
| pH (7.32–7.42) | 7.42 (7.38–7.46) |
| pCO2 (41–51mmHg) | 35 (31–39) |
| Bicarbonates (22–29 mmol/l) | 22.9 (21.1–25.4) |
| Lactic acid (<3.1 mmol/l) | 1.4 (1.2–1.9) |
| Troponin I (<40 ng/l) | 84.7 (26–398) |
| Troponin T (14 ng/l) | 24.6 (8–62.5) |
| Hs-troponin (<11 ng/l) | 33 (11.7–110.1) |
| Triglycerides (<150 mg/dl) | 184 (131–251) |
| ESR (<28 mm/h) | 53.5 (35–74) |
| Cholesterol (<190 mg/dl) | 111 (86–129) |
| Fibrinogen (170–420 mg/dl) | 582 (468–717) |
| INR | 1.2 (1.1–1.4) |
| aPTT (0.8–1.20 ratio) | 1.2 (1.1–1.3) |
| ATIII (79–120%) | 81 (73–93) |
| C3 (90-180 mg/dl) | 134 (89–153) |
| C4 (10-40 mg/dl) | 23.5 (13–32) |
| Ferritin (30-400 ng/ml) | 540 (287.5–915.5) |
| D-dimer (<500 ng/ml) | 2222 (1185.5–3821.5) |
| NT-proBNP (<400 pg/ml) | 3364.5 (873.9–9222.5) |
| BNP (<100 pg/ml) | 557.5 (135–1110) |
ALT: alanine aminotransferase; AST: aspartate aminotransferase; CRP: c-reactive protein; CPK: creatine phosphokinase; ESR: erythrocyte sedimentation rate; INR: international normalized ratio; APTT: activated partial thromboplastin time; AIII: antithrombin III; BNP: brain natriuretic peptide.
Demographic, clinical and laboratory manifestations of study population at onset
| Demographic characteristics | |
| Female/Male | 104/135 |
| Ethnicity, n (%) | |
| Caucasian ancestry | 190 (79.5) |
| African | 26 (10.9) |
| Asian | 17 (7.1) |
| Latin-American | 6 (2.5) |
| Age at onset, median (IQR), years | 8 (4–11) |
| Clinical manifestations | |
| At least one skin involvement, n (%) | 155 (64.9) |
| Maculopapular rash | 106 (44.4) |
| Multiform erythema | 23 (9.6) |
| Perineal erythema | 3 (1.3) |
| Scarlattinoform rash | 8 (3.4) |
| Palmoplantar erythema | 41 (17.2) |
| At least one mucosal involvement, n (%) | 154 (64.4) |
| Cheilitis | 97 (40.6) |
| Oral changes | 90 (37.7) |
| At least one ocular involvement, n (%) | 135 (56.5) |
| Conjunctivitis | 134 (56.1) |
| At least one neurological involvement, n (%) | 112 (46.9) |
| Headache | 42 (17.6) |
| Aseptic meningitis | 11 (4.6) |
| Irritability | 56 (23.4) |
| At least one gastrointestinal involvement, n (%) | 174 (72.8) |
| Diarrhoea | 105 (43.9) |
| Vomiting | 73 (30.6) |
| Abdominal pain | 112 (46.9) |
| Colecyts hydrops | 6 (2.5) |
| Acute pancreatitis | 3 (1.3) |
| Hepatomegaly | 30 (12.6) |
| Splenomegaly | 21 (8.8) |
| At least one lung involvement, n (%) | 80 (33.5) |
| Dyspnea | 27 (11.3) |
| Tachypnea | 28 (11.7) |
| Lobar pneumonia | 12 (5.0) |
| Interstitial pneumonia | 22 (9.2) |
| Oxygen supplementation | 37 (15.5) |
| Non-invasive/Invasive ventilation | 21 (8.8) |
| At least one heart involvement, n (%) | 169 (70.7) |
| Myocarditis | 64 (26.8) |
| Pericarditis | 32 (13.4) |
| Valvular insufficiency | 46 (19.3) |
| Coronary dilation | 22 (9.2) |
| Coronary aneurism | 8 (3.4) |
| Heart failure | 39 (16.4) |
| Hypotension/Non-cardiogenic shock | 46 (19.3) |
| Cardiac arrest | 2 (0.8) |
| At least one osteoarticular involvement, n (%) | 30 (12.6) |
| Arthritis/arthralgia | 19 (8.0) |
| Myositis | 4 (1.7) |
| Lymphadenopathy, n (%) | 72 (30.1) |
| Laboratory exams (normal values) | Median (IQR) |
| Leucocytes (5-19.5 103/mmc) | 10 795 (7750-13 615) |
| Neutrophils (1-9 103/mmc) | 8280 (5650-10450) |
| Lymphocytes (2.5-16.5 103/mmc) | 1070 (700-1950) |
| Monocytes (0.2-1.10 103/mmc) | 400 (220-780) |
| Haemoglobin (9-13.5 g/dL) | 11.3 (10.4-12.3) |
| Platelets (100-400/mmc) | 203 000 (121 000-275 000) |
| ALT (10-50 U/L) | 26 (18-44) |
| AST (28-41 U/L) | 34 (23-50) |
| GammaGT (39-308 U/L) | 23 (15-48) |
| Creatinine (0.17-0.43 mg/dL) | 0.5 (0.4-0.8) |
| Sodium (136-145mEq/L) | 134 (131-136) |
| Clorus (98-107mEq/L) | 99 (96-102) |
| Potassium (3.4-4.5mEq/L) | 4 (3.5-4.3) |
| CRP (<5mg/L) | 146.5 (97.0-227.1) |
| IgA (*mg/dL) | 102 (77-173) |
| IgG (*mg/dL) | 731.5 (591.5-993.5) |
| IgM (*mg/dL) | 98.5 (74-136.5) |
| Proteins (5.9-7.3 g/dL) | 6.3 (5.5-6.9) |
| Albumin (3.1-5.2 g/dL) | 3.1 (2.7-3.6) |
| CPK (39-308 U/L) | 66.5 (41-148) |
| pH (7.32–7.42) | 7.42 (7.38–7.46) |
| pCO2 (41–51mmHg) | 35 (31–39) |
| Bicarbonates (22–29 mmol/l) | 22.9 (21.1–25.4) |
| Lactic acid (<3.1 mmol/l) | 1.4 (1.2–1.9) |
| Troponin I (<40 ng/l) | 84.7 (26–398) |
| Troponin T (14 ng/l) | 24.6 (8–62.5) |
| Hs-troponin (<11 ng/l) | 33 (11.7–110.1) |
| Triglycerides (<150 mg/dl) | 184 (131–251) |
| ESR (<28 mm/h) | 53.5 (35–74) |
| Cholesterol (<190 mg/dl) | 111 (86–129) |
| Fibrinogen (170–420 mg/dl) | 582 (468–717) |
| INR | 1.2 (1.1–1.4) |
| aPTT (0.8–1.20 ratio) | 1.2 (1.1–1.3) |
| ATIII (79–120%) | 81 (73–93) |
| C3 (90-180 mg/dl) | 134 (89–153) |
| C4 (10-40 mg/dl) | 23.5 (13–32) |
| Ferritin (30-400 ng/ml) | 540 (287.5–915.5) |
| D-dimer (<500 ng/ml) | 2222 (1185.5–3821.5) |
| NT-proBNP (<400 pg/ml) | 3364.5 (873.9–9222.5) |
| BNP (<100 pg/ml) | 557.5 (135–1110) |
| Demographic characteristics | |
| Female/Male | 104/135 |
| Ethnicity, n (%) | |
| Caucasian ancestry | 190 (79.5) |
| African | 26 (10.9) |
| Asian | 17 (7.1) |
| Latin-American | 6 (2.5) |
| Age at onset, median (IQR), years | 8 (4–11) |
| Clinical manifestations | |
| At least one skin involvement, n (%) | 155 (64.9) |
| Maculopapular rash | 106 (44.4) |
| Multiform erythema | 23 (9.6) |
| Perineal erythema | 3 (1.3) |
| Scarlattinoform rash | 8 (3.4) |
| Palmoplantar erythema | 41 (17.2) |
| At least one mucosal involvement, n (%) | 154 (64.4) |
| Cheilitis | 97 (40.6) |
| Oral changes | 90 (37.7) |
| At least one ocular involvement, n (%) | 135 (56.5) |
| Conjunctivitis | 134 (56.1) |
| At least one neurological involvement, n (%) | 112 (46.9) |
| Headache | 42 (17.6) |
| Aseptic meningitis | 11 (4.6) |
| Irritability | 56 (23.4) |
| At least one gastrointestinal involvement, n (%) | 174 (72.8) |
| Diarrhoea | 105 (43.9) |
| Vomiting | 73 (30.6) |
| Abdominal pain | 112 (46.9) |
| Colecyts hydrops | 6 (2.5) |
| Acute pancreatitis | 3 (1.3) |
| Hepatomegaly | 30 (12.6) |
| Splenomegaly | 21 (8.8) |
| At least one lung involvement, n (%) | 80 (33.5) |
| Dyspnea | 27 (11.3) |
| Tachypnea | 28 (11.7) |
| Lobar pneumonia | 12 (5.0) |
| Interstitial pneumonia | 22 (9.2) |
| Oxygen supplementation | 37 (15.5) |
| Non-invasive/Invasive ventilation | 21 (8.8) |
| At least one heart involvement, n (%) | 169 (70.7) |
| Myocarditis | 64 (26.8) |
| Pericarditis | 32 (13.4) |
| Valvular insufficiency | 46 (19.3) |
| Coronary dilation | 22 (9.2) |
| Coronary aneurism | 8 (3.4) |
| Heart failure | 39 (16.4) |
| Hypotension/Non-cardiogenic shock | 46 (19.3) |
| Cardiac arrest | 2 (0.8) |
| At least one osteoarticular involvement, n (%) | 30 (12.6) |
| Arthritis/arthralgia | 19 (8.0) |
| Myositis | 4 (1.7) |
| Lymphadenopathy, n (%) | 72 (30.1) |
| Laboratory exams (normal values) | Median (IQR) |
| Leucocytes (5-19.5 103/mmc) | 10 795 (7750-13 615) |
| Neutrophils (1-9 103/mmc) | 8280 (5650-10450) |
| Lymphocytes (2.5-16.5 103/mmc) | 1070 (700-1950) |
| Monocytes (0.2-1.10 103/mmc) | 400 (220-780) |
| Haemoglobin (9-13.5 g/dL) | 11.3 (10.4-12.3) |
| Platelets (100-400/mmc) | 203 000 (121 000-275 000) |
| ALT (10-50 U/L) | 26 (18-44) |
| AST (28-41 U/L) | 34 (23-50) |
| GammaGT (39-308 U/L) | 23 (15-48) |
| Creatinine (0.17-0.43 mg/dL) | 0.5 (0.4-0.8) |
| Sodium (136-145mEq/L) | 134 (131-136) |
| Clorus (98-107mEq/L) | 99 (96-102) |
| Potassium (3.4-4.5mEq/L) | 4 (3.5-4.3) |
| CRP (<5mg/L) | 146.5 (97.0-227.1) |
| IgA (*mg/dL) | 102 (77-173) |
| IgG (*mg/dL) | 731.5 (591.5-993.5) |
| IgM (*mg/dL) | 98.5 (74-136.5) |
| Proteins (5.9-7.3 g/dL) | 6.3 (5.5-6.9) |
| Albumin (3.1-5.2 g/dL) | 3.1 (2.7-3.6) |
| CPK (39-308 U/L) | 66.5 (41-148) |
| pH (7.32–7.42) | 7.42 (7.38–7.46) |
| pCO2 (41–51mmHg) | 35 (31–39) |
| Bicarbonates (22–29 mmol/l) | 22.9 (21.1–25.4) |
| Lactic acid (<3.1 mmol/l) | 1.4 (1.2–1.9) |
| Troponin I (<40 ng/l) | 84.7 (26–398) |
| Troponin T (14 ng/l) | 24.6 (8–62.5) |
| Hs-troponin (<11 ng/l) | 33 (11.7–110.1) |
| Triglycerides (<150 mg/dl) | 184 (131–251) |
| ESR (<28 mm/h) | 53.5 (35–74) |
| Cholesterol (<190 mg/dl) | 111 (86–129) |
| Fibrinogen (170–420 mg/dl) | 582 (468–717) |
| INR | 1.2 (1.1–1.4) |
| aPTT (0.8–1.20 ratio) | 1.2 (1.1–1.3) |
| ATIII (79–120%) | 81 (73–93) |
| C3 (90-180 mg/dl) | 134 (89–153) |
| C4 (10-40 mg/dl) | 23.5 (13–32) |
| Ferritin (30-400 ng/ml) | 540 (287.5–915.5) |
| D-dimer (<500 ng/ml) | 2222 (1185.5–3821.5) |
| NT-proBNP (<400 pg/ml) | 3364.5 (873.9–9222.5) |
| BNP (<100 pg/ml) | 557.5 (135–1110) |
ALT: alanine aminotransferase; AST: aspartate aminotransferase; CRP: c-reactive protein; CPK: creatine phosphokinase; ESR: erythrocyte sedimentation rate; INR: international normalized ratio; APTT: activated partial thromboplastin time; AIII: antithrombin III; BNP: brain natriuretic peptide.
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