Bilateral Frosted Branch Angiitis Associated With Presumed Sarcoidosis: An Uncommon Challenging Presentation
Accept poster if oral is not possible ?
Yes
Purpose
To report a case of frosted-branch angiitis (FBA) associated with multisystemic changes, in a picture consistent with sarcoidosis
Methods
Retrospective chart review of clinical/laboratorial data, and of multimodal imaging
Results
A 23-year-old female presented with severe headache, polyarthralgia, and decreased vision. Ophthalmic examination revealed: VA of 20/60 (OD), counting fingers at 2m (OS); severe bilateral anterior chamber inflammation, with granulomatous KPs; bilateral optic disc edema, as well as, frosted-branch-like vascular sheathing, with diffuse perivascular hemorrhagic exudation. Sparse pre-retinal hemorrhages were also found, in addition to multiple yellowish supravenular exudates bilaterally, consistent with “candle-wax drippings”. Lumbar puncture revealed elevated opening pressure and lymphocytic pleocytosis. CNS imaging indicated intracranial hypertension, while serologies/rheumatologic markers were non-specific. Infectious causes were ruled out, and sarcoidosis was suspected based on ocular presentation, despite absence of pulmonary findings/biopsy confirmation at that stage.
Treatment with pulse intravenous methylprednisolone, followed by cyclophosphamide, led to visual improvement. Retinal hemorrhages resolved in a month, but vascular changes/macular ischemia persisted. Adalimumab therapy was initiated, and peripheral ischemia required laser. At three months, VA improved to 20/25 in OD and 20/80 in OS, with stable imaging findings and no active intraocular or systemic inflammation
Conclusion
FBA may be rarely associated with sarcoidosis, possibly paralleling severe systemic/CNS inflammation and thus requiring aggressive anti-inflammatory/immodulomodulatory therapy
Conflict of interest
No
1
Last name
FREIRE
Initials of first name(s)
BCC
Department
HOSPITAL SÃO GERALDO
City
BELO HORIZONTE
Country
Brazil
2
Last name
ROCHA
Initials of first name(s)
ACH
Department
Hospital São Geraldo/HC da Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, Brazil
City
Belo Horizonte
Country
Brazil
3
Last name
AZEVEDO
Initials of first name(s)
DOM
Department
Hospital São Geraldo/HC da Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, Brazil
City
Belo Horizonte
Country
Brazil
4
Last name
TANURE
Initials of first name(s)
LA
Department
Hospital São Geraldo/HC da Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, Brazil
City
Belo Horizonte
Country
Brazil
5
Last name
LARA
Initials of first name(s)
CM
Department
Hospital São Geraldo/HC da Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, Brazil 2 Fac
City
Belo Horizonte
Country
Brazil
6
Last name
ROCHA
Initials of first name(s)
EA
Department
1 Hospital São Geraldo/HC da Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, Brazil
City
Belo Horizonte
Country
Brazil
7
Last name
VASCONCELOS-SANTOS
Initials of first name(s)
DV
Department
1 Hospital São Geraldo/HC da UFMG / Faculdade de Medicina da UFMG, Belo Horizonte, Brazil
City
Belo Horizonte
Country
Brazil
This website uses cookies to ensure you get the best experience on our website.
Learn more