PMID- 36865980
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20230304
IS  - 2168-8184 (Print)
IS  - 2168-8184 (Electronic)
IS  - 2168-8184 (Linking)
VI  - 15
IP  - 1
DP  - 2023 Jan
TI  - Lupus Nephritis in an Adolescent Girl With Hyper-Immunoglobulin E.
PG  - e34332
LID - 10.7759/cureus.34332 [doi]
LID - e34332
AB  - We report the case of an adolescent girl with frequent hospital admissions for 
      severe eczematous skin rashes with recurrent epistaxis and chest infections. 
      Investigations revealed persistent severely elevated serum total immunoglobulin E 
      (IgE) levels but normal levels of other immunoglobulins, suggesting hyper-IgE 
      syndrome. The first skin biopsy revealed superficial dermatophytic dermatitis 
      (tinea corpora). Another biopsy performed after six months revealed a prominent 
      basement membrane with dermal mucin, suggesting an underlying autoimmune disease. 
      Her condition was complicated by proteinuria, hematuria, hypertension, and edema. 
      A kidney biopsy revealed class IV lupus nephritis, according to the International 
      Society of Nephrology/Renal Pathology Society (ISN/RPS). Based on the American 
      College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria, 
      she was diagnosed with systemic lupus erythematosus (SLE). She was first 
      administered with intravenous pulse methylprednisolone (600 mg/m(2)) for three 
      consecutive days, followed by oral prednisolone (40 mg/m(2)) daily, mycophenolate 
      mofetil tablets (600 mg/m(2)/dose) twice daily, hydroxychloroquine (200 mg) once 
      daily, and three classes of antihypertensive medications. She maintained normal 
      renal functions with no lupus morbidity for 24 months, then rapidly progressed to 
      end-stage kidney disease, and was then started on three to four sessions of 
      regular hemodialysis per week. Hyper-IgE is known to be a marker of immune 
      dysregulation as it facilitates the generation of immune complexes (ICs) that 
      mediate lupus nephritis and juvenile SLE. Regardless of the different factors 
      that are impacting the production of IgE, the present case illustrated that 
      juvenile patients with SLE may have increased IgE levels, indicating that higher 
      IgE levels might have a role in lupus pathogenesis and prognosis. The mechanisms 
      regarding the increased levels of IgE in subjects with lupus need further 
      investigation. Further studies are thus required to assess the incidence, 
      prognosis, and possible new specific management for hyper-IgE in juvenile SLE.
CI  - Copyright (c) 2023, Alahmadi et al.
FAU - Alahmadi, Ghadeer G
AU  - Alahmadi GG
AD  - Pediatrics, King Abdulaziz University Faculty of Medicine, Jeddah, SAU.
FAU - El-Desoky, Sherif M
AU  - El-Desoky SM
AD  - Pediatrics Nephrology, King Abdulaziz University Faculty of Medicine, Jeddah, 
      SAU.
FAU - Al Zahrani, Reem A
AU  - Al Zahrani RA
AD  - Pathology, King Abdulaziz University Faculty of Medicine, Jeddah, SAU.
FAU - Kari, Jameela A
AU  - Kari JA
AD  - Pediatrics Nephrology, King Abdulaziz University Faculty of Medicine, Jeddah, 
      SAU.
LA  - eng
PT  - Case Reports
DEP - 20230129
PL  - United States
TA  - Cureus
JT  - Cureus
JID - 101596737
PMC - PMC9972904
OTO - NOTNLM
OT  - hyper ige syndrome
OT  - immune complexes
OT  - immune dysregulation
OT  - lupus nephritis
OT  - systemic lupus erythematosus
COIS- The authors have declared that no competing interests exist.
EDAT- 2023/03/04 06:00
MHDA- 2023/03/04 06:01
PMCR- 2023/01/29
CRDT- 2023/03/03 02:38
PHST- 2023/01/29 00:00 [accepted]
PHST- 2023/03/03 02:38 [entrez]
PHST- 2023/03/04 06:00 [pubmed]
PHST- 2023/03/04 06:01 [medline]
PHST- 2023/01/29 00:00 [pmc-release]
AID - 10.7759/cureus.34332 [doi]
PST - epublish
SO  - Cureus. 2023 Jan 29;15(1):e34332. doi: 10.7759/cureus.34332. eCollection 2023 
      Jan.