Autoinflammation in Syndromic Hidradenitis Suppurativa: The Role of AIM2
Abstract
:1. Introduction
2. Materials and Methods
2.1. Patients
2.2. Gene Sequencing
3. Results
3.1. Clinical Features
3.2. Genetic Analyses
4. Discussion
5. Limitations and Strengths
Supplementary Materials
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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ID | Sex | Diagnosis | Comorbidities | Hidradenitis suppurativa | Pyoderma gangrenosum | Acne | Treatments | Follow-up | |||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Age at onset (years) | Involved sites | IHS4 | Age at onset (years) | Involved sites | PG Score | Age at onset (years) | Involved sites | ||||||
1 | M | PAPASH | pyogenic arthritis | 14 | axillae, groins, back, nuchal region, scalp | 30 | 18 | trunk | multi-lesional | 13 | face, back | rifampicin, doxycycline, azitromycine, lymecycline, clindamycin, adalimumab, anakinra, prednisone | CR |
2 | M | PASH/SAPHO | SAPHO | 43 | axillae, groins, anogenital area | 27 | 43 | lower limbs, trunk, upper limbs | disseminated | 17 | face, back | clindamycin, infliximab | CR |
3 | M | PASH/SAPHO | SAPHO | 42 | axillae, groins, anogenital area | 19 | 46 | lower limbs | localized | 16 | face | clindamycin, methotrexate, infliximab, adalimumab | CR |
4 | M | PASH | Obesity, BBS, osteoporosis, PPPP | 36 | axillae, groins, anogenital area | 24 | 36 | lower limbs, trunk, upper limbs | disseminated | 34 | face | trimetoprim-sulfametoxazole, azitromycine, cyclosporine, prednisone, adalimumab, ustekinumab, | CR |
5 | F | PASH | Obesity, BBS, myocardial infarction, type II DM, BP | 51 | axillae, groins, submammary folds, anogenital area | 23 | 51 | perianal region | localized | 15 | face | rifampicin, doxycycline, infliximab, adalimumab | CR |
6 | F | PASH | PCOS | 15 | Axillae, groin | 20 | 24 | Lower limbs | multi-lesional | 14 | Face, back | infliximab | CR |
7 | F | PASH/SAPHO | SAPHO | 15 | axillae, groins, anogenital region, nuchal region | 26 | 15 | trunk | multi-lesional | 13 | face | rifampicin, doxycycline, azitromycine, lymecycline, clindamycin, intravenous immunoglobulin, methotrexate, isotretinoin, adalimumab, anakinra, ustekinumab, secukinumab | NR |
8 | F | PAPASH | Ulcerative colitis, PCOS, pyogenic arthritis | 28 | submammary folds, groins, anogenital area | 22 | 36 | peristomal | localized | 15 | face, back | doxycycline, infliximab, surgery for HS | CR |
9 | M | PASH | Crohn disease, osteoporosis | 18 | axillae, groins, anogenital area, nuchal region | 23 | 19 | lower limbs | multi-lesional | 16 | face, back | azitromycine, trimetoprim-sulfametoxazole, ceftriaxone, ciprofloxacine, lymecycline, rifampicin, amoxicillin clavulanate, methylprednisolone, dapsone, cyclosporine, adalimumab, anakinra, | PR |
10 | F | PASH | Crohn disease, coeliac disease, myasthenia gravis, PCOS | 18 | axillae, nuchal region, submammary folds, groins, anogenital area | 19 | 44 | peristomal | localized | 17 | face, back | clindamycin, methotrexate, infliximab, adalimumab, surgery for HS | CR |
11 | F | PAPASH | FMF, PCOS, pyogenic arthritis | 16 | axillae, groins, anogenital area, intermammary and submammary folds | 20 | 16 | trunk | multi-lesional | 14 | face | azitromycine, doxycycline, dapsone, adalimumab, anakinra, colchicine | CR |
12 | M | PASH | Pilonidal cyst | 15 | axillae and anogenital | 21 | 18 | lower back | localized | 14 | Face, back | Azithromycin, infliximab, adalimumab | PR/CR |
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Moltrasio, C.; Cagliani, R.; Sironi, M.; Clerici, M.; Pontremoli, C.; Maronese, C.A.; Tricarico, P.M.; Crovella, S.; Marzano, A.V. Autoinflammation in Syndromic Hidradenitis Suppurativa: The Role of AIM2. Vaccines 2023, 11, 162. https://doi.org/10.3390/vaccines11010162
Moltrasio C, Cagliani R, Sironi M, Clerici M, Pontremoli C, Maronese CA, Tricarico PM, Crovella S, Marzano AV. Autoinflammation in Syndromic Hidradenitis Suppurativa: The Role of AIM2. Vaccines. 2023; 11(1):162. https://doi.org/10.3390/vaccines11010162
Chicago/Turabian StyleMoltrasio, Chiara, Rachele Cagliani, Manuela Sironi, Mario Clerici, Chiara Pontremoli, Carlo Alberto Maronese, Paola Maura Tricarico, Sergio Crovella, and Angelo Valerio Marzano. 2023. "Autoinflammation in Syndromic Hidradenitis Suppurativa: The Role of AIM2" Vaccines 11, no. 1: 162. https://doi.org/10.3390/vaccines11010162
APA StyleMoltrasio, C., Cagliani, R., Sironi, M., Clerici, M., Pontremoli, C., Maronese, C. A., Tricarico, P. M., Crovella, S., & Marzano, A. V. (2023). Autoinflammation in Syndromic Hidradenitis Suppurativa: The Role of AIM2. Vaccines, 11(1), 162. https://doi.org/10.3390/vaccines11010162