Table 1.
Risk factors of epidermolysis bullosa acquisita (EBA).
| Cases | Controls | |||||||||
|---|---|---|---|---|---|---|---|---|---|---|
| Disease | ICD10 code | N of eligible participants | N of Outcomes | Risk, % | N of eligible participants* | N of Outcomes | Risk, % | Risk difference (95% confidence interval), % | Hazard ratio (95% confidence interval) | P value |
| Overweight and obesity | E66 | 6,923,027 | 280 | 0.004 | 6,923,063 | 245 | 0.004 | 0.001, 0-0.001 | 1.257, 1.059-1.493 | 0.0088 |
| Type 2 diabetes mellitus | E11 | 5,637,675 | 240 | 0.004 | 5,637,665 | 255 | 0.005 | – | – | n.s. |
| Hypothyroidism, unspecified | E03.9 | 3,402,569 | 145 | 0.004 | 3,402,556 | 133 | 0.004 | – | – | n.s. |
| Malnutrition | E40-E46 | 919,769 | 46 | 0.005 | 919,811 | 33 | 0.004 | 0.001, 0-0.003 | 2.363, 1.505-3.71 | 0.0001 |
| Systemic lupus erythematosus | M32 | 231,329 | 29 | 0.013 | 231,352 | 10 | 0.004 | 0.008, 0.003-0.014 | 3.099, 1.466-6.552 | 0.0018 |
| Hypertensive diseases | I10-I16 | 7,840,452 | 284 | 0.004 | 7,840,405 | 307 | 0.004 | – | – | n.s. |
| Heart failure | I50 | 2,460,148 | 84 | 0.003 | 2,460,164 | 113 | 0.005 | – | – | n.s. |
| Atrial fibrillation and flutter | I48 | 2,440,224 | 84 | 0.003 | 2,440,208 | 113 | 0.005 | – | – | n.s. |
| Nonrheumatic mitral valve disorders | I34 | 1,549,166 | 64 | 0.004 | 1,549,175 | 57 | 0.004 | – | – | n.s. |
| Atherosclerosis | I70 | 1,246,293 | 57 | 0.005 | 1,246,328 | 50 | 0.004 | – | – | n.s. |
| Acute embolism and thrombosis of deep veins of lower extremity | I82.4 | 845,427 | 53 | 0.006 | 845,451 | 24 | 0.003 | 0.003, 0.001-0.005 | 2.628, 1.622-4.259 | < 0.0001 |
| Hypotension | I95 | 1,837,108 | 89 | 0.005 | 1,837,163 | 84 | 0.005 | 0, -0.001-0.002 | 1.395, 1,035-1,882 | 0.0282 |
| Cerebral infarction | I63 | 1,567,585 | 84 | 0.005 | 1,567,605 | 70 | 0.004 | 0.001, -0.001-0.002 | 1,494, 1,008-2,053 | 0.0126 |
| Diverticular disease of intestine | K57 | 2,337,084 | 112 | 0.005 | 2,337,112 | 132 | 0.006 | – | – | n.s. |
| Gastro-esophageal reflux disease | K21 | 6,973,564 | 295 | 0.004 | 6,973,597 | 251 | 0.004 | – | – | n.s. |
| Ulcer of esophagus | K22.1 | 127,391 | 11 | 0.009 | 127,404 | 10 | 0.008 | – | – | n.s. |
| Gastritis and duodenitis | K29 | 1,869,533 | 74 | 0.004 | 1,869,561 | 61 | 0.003 | – | – | n.s. |
| Stomatitis and related lesions | K12 | 624,417 | 71 | 0.011 | 624,467 | 14 | 0.002 | 0.009, 0.006-0.012 | 4.943, 2.785-8.773 | < 0.0001 |
| Diaphragmatic hernia | K44 | 1,329,587 | 66 | 0.005 | 1,329,616 | 70 | 0.005 | – | – | n.s. |
| Crohn’s disease | K50 | 294,555 | 20 | 0.007 | 294,562 | 11 | 0.004 | – | – | n.s. |
| Other chronic obstructive pulmonary disease | J44 | 2,317,942 | 72 | 0.003 | 2,317,939 | 114 | 0.005 | -0.002, -0.003-0.001 | 0.732 0.545-0.984 | 0.0378 |
| Sleep disorders | G47 | 5,970,685 | 251 | 0.004 | 5,970,732 | 216 | 0.004 | 0.001, 0-0.001 | 1.231, 1.026-1.477 | 0.0250 |
| Acute kidney failure and chronic kidney disease | N17-N19 | 3,557,269 | 169 | 0.005 | 3,557,299 | 158 | 0.004 | 0, -0.001-0.001 | 1.371, 1.103-1.704 | 0.0043 |
| Malignant melanoma of skin | C43 | 282,444 | 24 | 0.008 | 282,476 | 13 | 0.005 | – | – | n.s. |
| Malignant neoplasm of liver and intrahepatic bile ducts | C22 | 594,423 | 38 | 0.006 | 594,476 | 21 | 0.004 | 0.003, 0-0.005 | 3.037, 1.737-5.309 | < 0.0001 |
| Melanocytic nevi | D22 | 1,370,655 | 40 | 0.003 | 1,370,781 | 46 | 0.003 | – | – | n.s. |
| Other and unspecified malignant neoplasm of skin | C44 | 1,080,602 | 73 | 0.007 | 1,080,674 | 47 | 0.004 | 0.002, 0-0.004 | 1.504, 1.042-2.17 | 0.0282 |
| Anemia, unspecified | D64.9 | 4,097,465 | 190 | 0.005 | 4,097,521 | 148 | 0.004 | 0.001, 0-0.002 | 1.394, 1.124-1.728 | 0.0024 |
| Elevated white blood cell count | D72.82 | 1,554,558 | 94 | 0.006 | 1,554,612 | 61 | 0.004 | 0.002, 0.001-0.004 | 2.174, 1.57-3.009 | < 0.0001 |
| Purpura and other hemorrhagic conditions | D69 | 1,325,255 | 79 | 0.006 | 1,325,309 | 50 | 0.004 | 0.002, 0.001-0.004 | 1.977, 1.387-2.82 | 0.0001 |
| Iron deficiency anemia | D50 | 2,055,291 | 84 | 0.004 | 2,055,347 | 64 | 0.003 | 0.001, 0-0.002 | 1.473, 1.064-2.04 | 0.0190 |
| Age-related cataract | H25 | 1,663,835 | 111 | 0.007 | 1,663,880 | 83 | 0.005 | – | – | n.s. |
| Other cataract | H26 | 1,223,272 | 99 | 0.008 | 1,223,308 | 50 | 0.004 | 0.004, 0.002-0.006 | 1.663, 1.183-2.339 | 0.0031 |
| Visual disturbances and blindness | H53-H54 | 3,457,195 | 179 | 0.005 | 3,457,247 | 98 | 0.003 | 0.002, 0.001-0.003 | 1.956, 1.529-2.503 | < 0.0001 |
| Glaucoma | H40-H42 | 1,221,974 | 77 | 0.006 | 1,221,991 | 47 | 0.004 | – | – | n.s. |
| Dry eye syndrome | H04.12 | 908,659 | 57 | 0.006 | 908,695 | 35 | 0.004 | – | – | n.s. |
| Presbyopia | H52.4 | 878,532 | 77 | 0.009 | 878,566 | 41 | 0.005 | 0.004, 0.002-0.007 | 1.569, 1.074-2.294 | 0.0190 |
| Conjunctival scars | H11.2 | 11,556 | 14 | 0.121 | 11,573 | 0 | 0 | 0.121, 0.058-0.185% | n.a. | 0.0001 |
| Keratitis | H16 | 362,090 | 32 | 0.009 | 362,108 | 17 | 0.005 | – | – | n.s. |
| Disorders of choroid and retina | H30-H36 | 1,476,209 | 82 | 0.006 | 1,476,231 | 61 | 0.004 | – | – | n.s. |
| Lupus erythematosus | L93 | 92,859 | 28 | 0.03 | 92,904 | 10 | 0.011 | 0.019, 0.006-0.032 | 26.957, 3.666,198.223 | < 0.0001 |
| Pressure ulcer | L89 | 520,244 | 25 | 0.005 | 520,276 | 23 | 0.004 | 0, -0.002-0.003 | 1.828, 1.035-3.228 | 0.0348 |
| Pruritus | L29 | 1,719,598 | 180 | 0.01 | 1,719,763 | 56 | 0.003 | 0.007, 0.005-0.009 | 3.507, 2.598-4.735 | < 0.0001 |
| Seborrheic dermatitis | L21 | 667,636 | 44 | 0.007 | 667,680 | 14 | 0.002 | 0.004, 0.002-0.007 | 2.893, 1.585-5.283 | 0.0003 |
| Lichen simplex chronicus and prurigo | L28 | 257,992 | 48 | 0.019 | 258,031 | 11 | 0.004 | 0.014, 0.009-0.02 | 4.446, 2.309-8.561 | < 0.0001 |
| Urticaria | L50 | 1,072,233 | 69 | 0.006 | 1,072,291 | 18 | 0.002 | 0.005, 0.003- 0.006 | 4.094, 2.437-6.88 | < 0.0001 |
| Lichen planus | L43 | 74,580 | 21 | 0.028 | 74,609 | 10 | 0.013 | 0.015, 0-0.029 | 10.198, 2.391-43.499 | < 0.0001 |
| Chronic pain, not elsewhere classified | G89.2 | 4,199,569 | 152 | 0.004 | 4,199,663 | 169 | 0.004 | – | – | n.s. |
| Acute pain, not elsewhere classified | G89.1 | 1,261,295 | 61 | 0.005 | 1,261,333 | 34 | 0.003 | 0.002, 0.001-0.004 | 2.371, 1.554-3.617 | < 0.0001 |
| Polyneuropathy, unspecified | G62.9 | 1,013,058 | 74 | 0.007 | 1,013,111 | 40 | 0.004 | 0.003, 0.001-0.005 | 2.45, 1.647-3.644 | < 0.0001 |
| Benign prostatic hyperplasia | N40 | 1,593,540 | 70 | 0.004 | 1,593,550 | 84 | 0.005 | – | – | n.s. |
| Candidiasis | B37 | 1,933,646 | 107 | 0.006 | 1,933,718 | 46 | 0.002 | 0.003, 0.002-0.004 | 2.311, 1.636-3.266 | < 0.0001 |
| Dermatophytosis | B35 | 1,676,899 | 105 | 0.006 | 1,676,983 | 59 | 0.004 | 0.003, 0.001-0.004 | 1.565, 1.137-2.153 | 0.0056 |
| Sepsis, unspecified organism | A41.9 | 1,336,969 | 62 | 0.005 | 1,337,011 | 52 | 0.004 | 0.001, -0.001-0.002 | 1.935 1.333-2.808 | 0.0004 |
| Nicotine dependence | F17 | 5,515,751 | 196 | 0.004 | 5,515,748 | 213 | 0.004 | – | – | n.s. |
The risk of EBA development in individuals diagnosed with any one of the listed diseases cases to those without these diagnoses controls. Data shown displays results from measures of association excluding patients with EBA prior to the time window. For the Hazard ratio. Kaplan-Meier Analysis again excluding patients with outcome prior to the time window with Log-Rank Test was performed. N, number; n.s., not significant; n.a., not applicable; Diseases where presence of any of the diseases increases the risk are highlighted in bold. Diseases where presence of the indicated disease decreases the risk are highlighted in blue and bold. Diseases that do not impose a risk for subsequent EBA development are displayed in gray. Please note that propensity score matching is re-run with each outcomes analysis so that the analysis uses the most current data available on the TriNetX network. Analysis was performed from October 3rd to 8th, 2022.