| Patient characteristic . | Value . |
|---|---|
| Year of diagnosis, median (IQR) | October 1994 (July 1992–October 2001) |
| Follow-up duration from diagnosis, median (IQR), months | 42.5 (22.1–73.6) |
| Delay to diagnosis, median (IQR), months | 3.9 (1.8–7.2) |
| Age at first treatment, median (IQR), years | 7.2 (4.6–10.6) |
| Female gender, n (%) | 213 (69.4) |
| Race/ethnicity, n (%) | |
| Caucasian | 217 (70.7) |
| African American | 32 (10.4) |
| Hispanic | 17 (5.5) |
| Other | 41 (13.4) |
| ANA positive (n = 288), n (%) | 206 (71.5) |
| Myositis autoantibodies (n = 305)a, n (%) | |
| Any MSA | 247 (81.0) |
| Any MSA with any MAA | 19 (6.2) |
| Anti-p155/140 (TIF1) | 118 (38.7) |
| Anti-MJ (NXP2) | 86 (28.2) |
| Anti-MDA5 | 25 (8.2) |
| Other MSAs (ARS, Mi-2, HMGCR) | 18 (5.9) |
| Any MAA | 29 (9.5) |
| MSA/MAA negative | 47 (15.4) |
| Onset severity, n (%) | |
| Mild | 34 (11.1) |
| Moderate | 188 (61.2) |
| Severe/very severe | 85 (27.7) |
| Clinical features (ever present), n (%) | |
| Any GI, pulmonary, or cardiac signs/symptoms | 234 (76.2) |
| Joint contractures | 183 (59.8) |
| Photosensitivity | 151 (50.3) |
| Arthritis | 144 (46.9) |
| Distal weakness (n = 296) | 136 (45.9) |
| Any pulmonary or cardiac signs/symptoms | 126 (41.0) |
| Dysphagia | 121 (39.4) |
| Weight loss | 107 (34.9) |
| Dysphonia | 101 (33.1) |
| Calcinosis | 93 (30.3) |
| Any GI/cutaneous ulceration | 62 (20.2) |
| Lipodystrophy | 28 (9.1) |
| Interstitial lung disease | 15 (4.9) |
| Organ symptom score at diagnosis, median (IQR) | |
| Total symptom score at diagnosis | 0.23 (0.15–0.32) |
| Muscle symptom score | 0.33 (0.25–0.5) |
| Skin symptom score | 0.28 (0.17–0.35) |
| Skeletal symptom score | 0.5 (0–0.5) |
| Constitutional symptom score | 0.25 (0.25–0.5) |
| Gastrointestinal symptom score | 0 (0–0.11) |
| Pulmonary symptom score | 0 (0–0.17) |
| Cardiac symptom score | 0 (0–0) |
| Highest values of serum muscle enzymes, median (IQR), U/l | |
| CK | 721 (282–3593) |
| Aldolase | 11.7 (8.1–19.2) |
| AST | 83 (47–161) |
| ALT | 71 (34–133) |
| LDH | 430 (299–690) |
| Environmental factors within 6 months diagnosis, n (%) | |
| Documented infection | 59 (19.2) |
| Site of infection | |
| Respiratory | 43 (14.0) |
| Mucocutaneous | 4 (1.3) |
| Gastrointestinal | 7 (2.3) |
| Immunization | 56 (18.2) |
| Medication received | 48 (15.6) |
| History of sun exposure | 24 (7.8) |
| UV index in 30 days before illness onset based on residential locationb, n (%) | |
| Average UV index, median (IQR) | 4.4 (2.1–6.2) |
| Highest UV index, median (IQR) | 6.7 (3.4–8.6) |
| United States geoclimatic zone at illness onset based on residential location (n = 283)b, n (%) | |
| Northeast | 82 (29.0) |
| South | 41 (15.5) |
| Northwest | 37 (13.1) |
| Central | 35 (12.4) |
| Southeast | 30 (10.6) |
| West | 22 (7.8) |
| East north central | 19 (6.7) |
| Southwest | 9 (3.2) |
| West north central | 5 (1.8) |
| Hawaii | 2 (0.7) |
| Alaska | 1 (0.4) |
| History of hospitalization (n = 298), n (%) | 161 (54.0) |
| History of wheelchair use (n =303), n (%) | 49 (16.2) |
| History of device use (n = 302), n (%) | 27 (8.9) |
| Medication received, n (%) | |
| Oral prednisone | 305 (99.3) |
| Methotrexate | 216 (70.4) |
| i.v. methylprednisolone | 173 (56.4) |
| Antimalarial drugs | 149 (48.5) |
| i.v. immunoglobulin | 117 (38.1) |
| Other DMARDs | 62 (20.2) |
| Cytotoxic/biologics | 38 (12.4) |
| Time from first symptoms to first treatment, median (IQR), months | 4.0 (2.0–8.0) |
| History of medication escalation within 18 months of treatment initiation, n (%) | 128 (41.7) |
| Patient characteristic . | Value . |
|---|---|
| Year of diagnosis, median (IQR) | October 1994 (July 1992–October 2001) |
| Follow-up duration from diagnosis, median (IQR), months | 42.5 (22.1–73.6) |
| Delay to diagnosis, median (IQR), months | 3.9 (1.8–7.2) |
| Age at first treatment, median (IQR), years | 7.2 (4.6–10.6) |
| Female gender, n (%) | 213 (69.4) |
| Race/ethnicity, n (%) | |
| Caucasian | 217 (70.7) |
| African American | 32 (10.4) |
| Hispanic | 17 (5.5) |
| Other | 41 (13.4) |
| ANA positive (n = 288), n (%) | 206 (71.5) |
| Myositis autoantibodies (n = 305)a, n (%) | |
| Any MSA | 247 (81.0) |
| Any MSA with any MAA | 19 (6.2) |
| Anti-p155/140 (TIF1) | 118 (38.7) |
| Anti-MJ (NXP2) | 86 (28.2) |
| Anti-MDA5 | 25 (8.2) |
| Other MSAs (ARS, Mi-2, HMGCR) | 18 (5.9) |
| Any MAA | 29 (9.5) |
| MSA/MAA negative | 47 (15.4) |
| Onset severity, n (%) | |
| Mild | 34 (11.1) |
| Moderate | 188 (61.2) |
| Severe/very severe | 85 (27.7) |
| Clinical features (ever present), n (%) | |
| Any GI, pulmonary, or cardiac signs/symptoms | 234 (76.2) |
| Joint contractures | 183 (59.8) |
| Photosensitivity | 151 (50.3) |
| Arthritis | 144 (46.9) |
| Distal weakness (n = 296) | 136 (45.9) |
| Any pulmonary or cardiac signs/symptoms | 126 (41.0) |
| Dysphagia | 121 (39.4) |
| Weight loss | 107 (34.9) |
| Dysphonia | 101 (33.1) |
| Calcinosis | 93 (30.3) |
| Any GI/cutaneous ulceration | 62 (20.2) |
| Lipodystrophy | 28 (9.1) |
| Interstitial lung disease | 15 (4.9) |
| Organ symptom score at diagnosis, median (IQR) | |
| Total symptom score at diagnosis | 0.23 (0.15–0.32) |
| Muscle symptom score | 0.33 (0.25–0.5) |
| Skin symptom score | 0.28 (0.17–0.35) |
| Skeletal symptom score | 0.5 (0–0.5) |
| Constitutional symptom score | 0.25 (0.25–0.5) |
| Gastrointestinal symptom score | 0 (0–0.11) |
| Pulmonary symptom score | 0 (0–0.17) |
| Cardiac symptom score | 0 (0–0) |
| Highest values of serum muscle enzymes, median (IQR), U/l | |
| CK | 721 (282–3593) |
| Aldolase | 11.7 (8.1–19.2) |
| AST | 83 (47–161) |
| ALT | 71 (34–133) |
| LDH | 430 (299–690) |
| Environmental factors within 6 months diagnosis, n (%) | |
| Documented infection | 59 (19.2) |
| Site of infection | |
| Respiratory | 43 (14.0) |
| Mucocutaneous | 4 (1.3) |
| Gastrointestinal | 7 (2.3) |
| Immunization | 56 (18.2) |
| Medication received | 48 (15.6) |
| History of sun exposure | 24 (7.8) |
| UV index in 30 days before illness onset based on residential locationb, n (%) | |
| Average UV index, median (IQR) | 4.4 (2.1–6.2) |
| Highest UV index, median (IQR) | 6.7 (3.4–8.6) |
| United States geoclimatic zone at illness onset based on residential location (n = 283)b, n (%) | |
| Northeast | 82 (29.0) |
| South | 41 (15.5) |
| Northwest | 37 (13.1) |
| Central | 35 (12.4) |
| Southeast | 30 (10.6) |
| West | 22 (7.8) |
| East north central | 19 (6.7) |
| Southwest | 9 (3.2) |
| West north central | 5 (1.8) |
| Hawaii | 2 (0.7) |
| Alaska | 1 (0.4) |
| History of hospitalization (n = 298), n (%) | 161 (54.0) |
| History of wheelchair use (n =303), n (%) | 49 (16.2) |
| History of device use (n = 302), n (%) | 27 (8.9) |
| Medication received, n (%) | |
| Oral prednisone | 305 (99.3) |
| Methotrexate | 216 (70.4) |
| i.v. methylprednisolone | 173 (56.4) |
| Antimalarial drugs | 149 (48.5) |
| i.v. immunoglobulin | 117 (38.1) |
| Other DMARDs | 62 (20.2) |
| Cytotoxic/biologics | 38 (12.4) |
| Time from first symptoms to first treatment, median (IQR), months | 4.0 (2.0–8.0) |
| History of medication escalation within 18 months of treatment initiation, n (%) | 128 (41.7) |
Variables in this table were as defined in prior studies [10–12, 16]. aFor this study, MSA and MAA were examined separately. Other MSAs included: anti-Mi-2 (n = 9), anti-ARS (n = 7), anti-HMGCR (n = 2). MAAs present in the population included: anti-Ro (n = 15), anti-PM-Scl (n = 7), anti-U1RNP(n = 4), anti-SUMO (n = 1), anti-Th (n = 1), anti-U3RNP (n = 1) and anti-U5RNP (n = 1). bUV index in 30 days before illness onset and US geoclimatic regions at illness onset were as defined in prior study [13] based on National Oceanic and Atmospheric Administration (NOAA) definitions and data. US geoclimatic zones defined by NOAA at https://www.ncdc.noaa.gov/monitoring-references/maps/us-climate-regions.php, (ftp://ftp.cpc.ncep.noaa.gov/long/uv/cities). ALT: alanine aminotransferase; AST: aspartate aminotransferase; ARS: aminoacyl-transfer RNA synthetase; CK: creatine kinase; GI: gastrointestinal; HMGCR: 3-hydroxy-3-methyl-glutaryl-coenzyme A reductase; IQR: interquartile range; i.v.: intravenous; LDH: lactate dehydrogenase; MAAs: myositis associated autoantibodies; MDA5: melanoma differentiation-associated protein 5; MSAs: myositis specific autoantibodies; NXP2: nuclear matrix protein 2; TIF1: transcriptional intermediary factor 1; UV: ultraviolet.
| Patient characteristic . | Value . |
|---|---|
| Year of diagnosis, median (IQR) | October 1994 (July 1992–October 2001) |
| Follow-up duration from diagnosis, median (IQR), months | 42.5 (22.1–73.6) |
| Delay to diagnosis, median (IQR), months | 3.9 (1.8–7.2) |
| Age at first treatment, median (IQR), years | 7.2 (4.6–10.6) |
| Female gender, n (%) | 213 (69.4) |
| Race/ethnicity, n (%) | |
| Caucasian | 217 (70.7) |
| African American | 32 (10.4) |
| Hispanic | 17 (5.5) |
| Other | 41 (13.4) |
| ANA positive (n = 288), n (%) | 206 (71.5) |
| Myositis autoantibodies (n = 305)a, n (%) | |
| Any MSA | 247 (81.0) |
| Any MSA with any MAA | 19 (6.2) |
| Anti-p155/140 (TIF1) | 118 (38.7) |
| Anti-MJ (NXP2) | 86 (28.2) |
| Anti-MDA5 | 25 (8.2) |
| Other MSAs (ARS, Mi-2, HMGCR) | 18 (5.9) |
| Any MAA | 29 (9.5) |
| MSA/MAA negative | 47 (15.4) |
| Onset severity, n (%) | |
| Mild | 34 (11.1) |
| Moderate | 188 (61.2) |
| Severe/very severe | 85 (27.7) |
| Clinical features (ever present), n (%) | |
| Any GI, pulmonary, or cardiac signs/symptoms | 234 (76.2) |
| Joint contractures | 183 (59.8) |
| Photosensitivity | 151 (50.3) |
| Arthritis | 144 (46.9) |
| Distal weakness (n = 296) | 136 (45.9) |
| Any pulmonary or cardiac signs/symptoms | 126 (41.0) |
| Dysphagia | 121 (39.4) |
| Weight loss | 107 (34.9) |
| Dysphonia | 101 (33.1) |
| Calcinosis | 93 (30.3) |
| Any GI/cutaneous ulceration | 62 (20.2) |
| Lipodystrophy | 28 (9.1) |
| Interstitial lung disease | 15 (4.9) |
| Organ symptom score at diagnosis, median (IQR) | |
| Total symptom score at diagnosis | 0.23 (0.15–0.32) |
| Muscle symptom score | 0.33 (0.25–0.5) |
| Skin symptom score | 0.28 (0.17–0.35) |
| Skeletal symptom score | 0.5 (0–0.5) |
| Constitutional symptom score | 0.25 (0.25–0.5) |
| Gastrointestinal symptom score | 0 (0–0.11) |
| Pulmonary symptom score | 0 (0–0.17) |
| Cardiac symptom score | 0 (0–0) |
| Highest values of serum muscle enzymes, median (IQR), U/l | |
| CK | 721 (282–3593) |
| Aldolase | 11.7 (8.1–19.2) |
| AST | 83 (47–161) |
| ALT | 71 (34–133) |
| LDH | 430 (299–690) |
| Environmental factors within 6 months diagnosis, n (%) | |
| Documented infection | 59 (19.2) |
| Site of infection | |
| Respiratory | 43 (14.0) |
| Mucocutaneous | 4 (1.3) |
| Gastrointestinal | 7 (2.3) |
| Immunization | 56 (18.2) |
| Medication received | 48 (15.6) |
| History of sun exposure | 24 (7.8) |
| UV index in 30 days before illness onset based on residential locationb, n (%) | |
| Average UV index, median (IQR) | 4.4 (2.1–6.2) |
| Highest UV index, median (IQR) | 6.7 (3.4–8.6) |
| United States geoclimatic zone at illness onset based on residential location (n = 283)b, n (%) | |
| Northeast | 82 (29.0) |
| South | 41 (15.5) |
| Northwest | 37 (13.1) |
| Central | 35 (12.4) |
| Southeast | 30 (10.6) |
| West | 22 (7.8) |
| East north central | 19 (6.7) |
| Southwest | 9 (3.2) |
| West north central | 5 (1.8) |
| Hawaii | 2 (0.7) |
| Alaska | 1 (0.4) |
| History of hospitalization (n = 298), n (%) | 161 (54.0) |
| History of wheelchair use (n =303), n (%) | 49 (16.2) |
| History of device use (n = 302), n (%) | 27 (8.9) |
| Medication received, n (%) | |
| Oral prednisone | 305 (99.3) |
| Methotrexate | 216 (70.4) |
| i.v. methylprednisolone | 173 (56.4) |
| Antimalarial drugs | 149 (48.5) |
| i.v. immunoglobulin | 117 (38.1) |
| Other DMARDs | 62 (20.2) |
| Cytotoxic/biologics | 38 (12.4) |
| Time from first symptoms to first treatment, median (IQR), months | 4.0 (2.0–8.0) |
| History of medication escalation within 18 months of treatment initiation, n (%) | 128 (41.7) |
| Patient characteristic . | Value . |
|---|---|
| Year of diagnosis, median (IQR) | October 1994 (July 1992–October 2001) |
| Follow-up duration from diagnosis, median (IQR), months | 42.5 (22.1–73.6) |
| Delay to diagnosis, median (IQR), months | 3.9 (1.8–7.2) |
| Age at first treatment, median (IQR), years | 7.2 (4.6–10.6) |
| Female gender, n (%) | 213 (69.4) |
| Race/ethnicity, n (%) | |
| Caucasian | 217 (70.7) |
| African American | 32 (10.4) |
| Hispanic | 17 (5.5) |
| Other | 41 (13.4) |
| ANA positive (n = 288), n (%) | 206 (71.5) |
| Myositis autoantibodies (n = 305)a, n (%) | |
| Any MSA | 247 (81.0) |
| Any MSA with any MAA | 19 (6.2) |
| Anti-p155/140 (TIF1) | 118 (38.7) |
| Anti-MJ (NXP2) | 86 (28.2) |
| Anti-MDA5 | 25 (8.2) |
| Other MSAs (ARS, Mi-2, HMGCR) | 18 (5.9) |
| Any MAA | 29 (9.5) |
| MSA/MAA negative | 47 (15.4) |
| Onset severity, n (%) | |
| Mild | 34 (11.1) |
| Moderate | 188 (61.2) |
| Severe/very severe | 85 (27.7) |
| Clinical features (ever present), n (%) | |
| Any GI, pulmonary, or cardiac signs/symptoms | 234 (76.2) |
| Joint contractures | 183 (59.8) |
| Photosensitivity | 151 (50.3) |
| Arthritis | 144 (46.9) |
| Distal weakness (n = 296) | 136 (45.9) |
| Any pulmonary or cardiac signs/symptoms | 126 (41.0) |
| Dysphagia | 121 (39.4) |
| Weight loss | 107 (34.9) |
| Dysphonia | 101 (33.1) |
| Calcinosis | 93 (30.3) |
| Any GI/cutaneous ulceration | 62 (20.2) |
| Lipodystrophy | 28 (9.1) |
| Interstitial lung disease | 15 (4.9) |
| Organ symptom score at diagnosis, median (IQR) | |
| Total symptom score at diagnosis | 0.23 (0.15–0.32) |
| Muscle symptom score | 0.33 (0.25–0.5) |
| Skin symptom score | 0.28 (0.17–0.35) |
| Skeletal symptom score | 0.5 (0–0.5) |
| Constitutional symptom score | 0.25 (0.25–0.5) |
| Gastrointestinal symptom score | 0 (0–0.11) |
| Pulmonary symptom score | 0 (0–0.17) |
| Cardiac symptom score | 0 (0–0) |
| Highest values of serum muscle enzymes, median (IQR), U/l | |
| CK | 721 (282–3593) |
| Aldolase | 11.7 (8.1–19.2) |
| AST | 83 (47–161) |
| ALT | 71 (34–133) |
| LDH | 430 (299–690) |
| Environmental factors within 6 months diagnosis, n (%) | |
| Documented infection | 59 (19.2) |
| Site of infection | |
| Respiratory | 43 (14.0) |
| Mucocutaneous | 4 (1.3) |
| Gastrointestinal | 7 (2.3) |
| Immunization | 56 (18.2) |
| Medication received | 48 (15.6) |
| History of sun exposure | 24 (7.8) |
| UV index in 30 days before illness onset based on residential locationb, n (%) | |
| Average UV index, median (IQR) | 4.4 (2.1–6.2) |
| Highest UV index, median (IQR) | 6.7 (3.4–8.6) |
| United States geoclimatic zone at illness onset based on residential location (n = 283)b, n (%) | |
| Northeast | 82 (29.0) |
| South | 41 (15.5) |
| Northwest | 37 (13.1) |
| Central | 35 (12.4) |
| Southeast | 30 (10.6) |
| West | 22 (7.8) |
| East north central | 19 (6.7) |
| Southwest | 9 (3.2) |
| West north central | 5 (1.8) |
| Hawaii | 2 (0.7) |
| Alaska | 1 (0.4) |
| History of hospitalization (n = 298), n (%) | 161 (54.0) |
| History of wheelchair use (n =303), n (%) | 49 (16.2) |
| History of device use (n = 302), n (%) | 27 (8.9) |
| Medication received, n (%) | |
| Oral prednisone | 305 (99.3) |
| Methotrexate | 216 (70.4) |
| i.v. methylprednisolone | 173 (56.4) |
| Antimalarial drugs | 149 (48.5) |
| i.v. immunoglobulin | 117 (38.1) |
| Other DMARDs | 62 (20.2) |
| Cytotoxic/biologics | 38 (12.4) |
| Time from first symptoms to first treatment, median (IQR), months | 4.0 (2.0–8.0) |
| History of medication escalation within 18 months of treatment initiation, n (%) | 128 (41.7) |
Variables in this table were as defined in prior studies [10–12, 16]. aFor this study, MSA and MAA were examined separately. Other MSAs included: anti-Mi-2 (n = 9), anti-ARS (n = 7), anti-HMGCR (n = 2). MAAs present in the population included: anti-Ro (n = 15), anti-PM-Scl (n = 7), anti-U1RNP(n = 4), anti-SUMO (n = 1), anti-Th (n = 1), anti-U3RNP (n = 1) and anti-U5RNP (n = 1). bUV index in 30 days before illness onset and US geoclimatic regions at illness onset were as defined in prior study [13] based on National Oceanic and Atmospheric Administration (NOAA) definitions and data. US geoclimatic zones defined by NOAA at https://www.ncdc.noaa.gov/monitoring-references/maps/us-climate-regions.php, (ftp://ftp.cpc.ncep.noaa.gov/long/uv/cities). ALT: alanine aminotransferase; AST: aspartate aminotransferase; ARS: aminoacyl-transfer RNA synthetase; CK: creatine kinase; GI: gastrointestinal; HMGCR: 3-hydroxy-3-methyl-glutaryl-coenzyme A reductase; IQR: interquartile range; i.v.: intravenous; LDH: lactate dehydrogenase; MAAs: myositis associated autoantibodies; MDA5: melanoma differentiation-associated protein 5; MSAs: myositis specific autoantibodies; NXP2: nuclear matrix protein 2; TIF1: transcriptional intermediary factor 1; UV: ultraviolet.
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