Ingrid Ruiz-Ordoñez, Colombia
Fundación Valle del Lili, Universidad Icesi GIRATPresenter of 3 Presentations
CLINICAL, SEROLOGICAL, IMMUNOLOGICAL AND HISTOPATHOLOGICAL CHARACTERIZATION OF PATIENTS WITH FIBROSING FRONTAL ALOPECIA
Abstract
Background and Aims
Frontal fibrosing alopecia (FFA) is a rare type of cicatricial alopecia. The specific mechanisms of development of FFA remain unknown. We previously proposed an autoimmune origin (Med Hypotheses2019;124). Here we describe the clinical and immunohistochemical profile of patients with FFA.
Methods
This was a retrospective cohort of 11 patients with FFA diagnosed by scalp biopsy. To evaluate the expression of immunological markers in 10 available scalp biopsies, we assessed by immunohistochemistry the following markers: B2 microglobulin, HLA class I and II, CD20, CD25, CD3, CD4, and CD8.
Results
All patients were female. Mean age at FFA diagnosis was 55.9 ± 9.51 years. Four (36.36%) patients had SS, and the same number had hypothyroidism. Five (45.45%) patients had positive antinuclear antibodies. In most of the samples, we observed expression of B2 microglobulin, HLA class I and II in the hair follicle. Only one (10%) patient showed CD20+ expression, while four (40%) patients presented infiltrating T lymphocytes (CD3+), including inverted relation among CD4/CD8 in two cases (20%). In 2 patients with SS, infiltrate expression was seen after 2- and 5-years of diagnosis. A patient with a recent diagnosis of SS did not show expression of any marker.
Conclusions
Our findings show a possible immune origin of FFA, represented by lymphocytic infiltration, expression of B2 immunoglobulin, HLA class I, and II causing impairment in the immune privilege of the hair follicle. The association of FFA with SS and hypothyroidism arises the possibility of an autoimmune origin.
LYMPHADENOPATHIES IN LONG-STANDING SYSTEMIC LUPUS ERYTHEMATOSUS AND THE ROLE OF MAINTENANCE THERAPY WITH RITUXIMAB.
Abstract
Background and Aims
Systemic Lupus Erythematosus (SLE) is a chronic multisystemic autoimmune inflammatory disease that may cause lymphadenopathies. The present study aimed to report the etiology of lymphadenopathies and the possible role of long-standing rituximab treatment on the expression of B cell-stimulating factors in lymph nodes.
Methods
This a retrospective cohort of 205 patients with SLE, of which 13 patients (6.3%) had lymphadenopathy. Immunohistochemistry was performed in 4 available cases of follicular hyperplasia to compare the expression of BAFF, BAFFR, and BCMA between patients who received Rituximab and those who did not.
Results
The etiology of 13 lymph nodes was as follows: follicular hyperplasia in 7 (53.85%) patients, Kikuchi's syndrome in 3 (23.08%), metastasis of papillary necrotizing lymphadenitis in 1 (7.69%), follicular B-cell lymphoma in 1 (7.69%) and tuberculosis in 1 (7.69%). Three patients presenting lymphadenopathies were in remission for several years and were receiving monotherapy with rituximab. Immunohistochemical study in one of these patients showed BAFF overexpression in the follicles and moderate expression of BAFF-R confined to the mantle zone (Figure 1). No significant differences were seen on the expression of BAFF, BAFF-R and BCMA when comparing with patients who did not received Rituximab.
Conclusions
The most common cause of lymphadenopathies in our series was follicular hyperplasia. Immunohistochemistry of one patient who received Rituximab showed overexpression of BAFF and moderate expression of BAFF-R. This finding could suggest that lymph node enlargement after CD20 depletion therapy may be mediated by compensatory expression of BAFF and its receptors.
SYSTEMIC LUPUS ERYTHEMATOSUS IN THE INTENSIVE CARE UNIT: AN OBSERVATIONAL STUDY
Abstract
Background and Aims
Systemic lupus erythematosus (SLE) is an autoimmune disease with complex pathophysiologic mechanisms and diverse clinical manifestations. SLE is a frequent cause of intensive care unit (ICU) admissions. The present study aimed to describe clinical and immunological variables in patients with SLE admitted to ICU at a single center in Cali, Colombia.
Methods
An observational, retrospective study was performed between 2011-2019 by reviewing the clinical records of patients with SLE admitted to ICU.
Results
182 patients were included, with a total of 252 admissions to the ICU; 154 (84.6%) were women with a median age of 35 [24-48] years. The main comorbidity and manifestation of the disease at admission was renal involvement in 66 (36.2%) and 89 (48.9%) patients, respectively, with a requirement for renal replacement therapy in 43 (23.63%) patients. The leading cause of medical admission to the ICU was an infection in 73/252 entries, followed by lupus flare in 44/252. The median days of stay in the ICU were 5 (3-11) with a SLEDAI of 9 (2-7), The Acute Physiology, Age and Chronic Health Evaluation (APACHE II) of 14 (10-17) and Sequential Organ Failure Assessment (SOFA) of 3 (2-5). The last two scores were higher in non-survivors patients (p=0.0004 and p=0.0001, respectively). Thirty-five (19.23%) patients died being infections the leading cause (24/35).
Conclusions
The leading cause of ICU admission was infection; patients with higher APACHE and SOFA scores were more susceptible to poor outcomes. More than 50% of deaths were attributable to infection.