scholarly journals Vaskulitis pada Lupus Eritematosus Sistemik

2020 ◽  
Vol 9 (1) ◽  
pp. 127
Author(s):  
Zikry Aulia Hidayat ◽  
Raveinal Raveinal
Keyword(s):  
Lupus Erythematosus ◽  
Vessel Wall ◽  
Cutaneous Lupus Erythematosus ◽  
Discoid Lupus Erythematosus ◽  
Intravenous Methylprednisolone ◽  
Once Daily ◽  
Pmn Leukocyte ◽  
Per Oral ◽  
Complex Autoimmune Disease ◽  
Cutaneous Lupus

AbstrakLupus Eritematosus Sistemik (LES) merupakan penyakit autoimun kompleks yang menyerang berbagai sistem tubuh. Salah satu manifestasi LES adalah vaskulitis, yaitu inflamasi pada dinding pembuluh darah. Vaskulitis sekunder akibat LES terjadi pada beberapa pasien LES dengan gambaran yang bervariasi. Gambaran vaskulitis yang tersering pada LES adalah vaskulitis kutaneus. Dilaporkan seorang perempuan 26 tahun yang telah dikenal dengan LES, datang dengan keluhan bintik-bintik merah di kedua tungkai dan lengan yang tidak gatal dan tidak nyeri. Dilakukan biopsi pada lesi kulit dengan hasil tampak pada jaringan kulit dengan epidermis yang mengalami atrofi, hyperkeratosis, ketotik plague, terdapat daerah dengan penebalan membrana basalis. Dibawah dermis tampak sebukan sel radang perivasikuler, terdapat limfosit dan beberapa leukosit PMN. Gambaran tersebut sesuai dengan gambaran discoid lupus erythematosus dan vaskulitis. Discoid lupus erythematosus merupakan salah satu bentuk dari lupus eritematosus kutaneus dan merupakan bentuk yang tersering. Pada kasus ini vaskulitis muncul menunjukkan kambuh atau aktifnya penyakit LES yang telah diderita pasien selama 2 tahun. Hal tersebut ditunjukkan dengan jumlah skor MEX SLEDAI 6 dan skor SLEDAI 12. Pasien diberikan terapi dengan metil prednisolon intravena 2x125 mg selama 3 hari serta hidroksiklorokuin 1x200 mg per oral. Respon yang baik terlihat pada hari rawatan keenam dengan berkurangnya manifestasi vaskulitis secara signifikan.Kata kunci: lupus eritematosus sistemik, vaskulitisAbstractSystemic lupus erythematosus (SLE) is a complex autoimmune disease involving many systems in one’s body. One of SLE manifestations is vasculitis, an inflammation of the vessel wall. Secondary vasculitis caused by SLE happened in few SLE patients with various manifestations. The most common SLE vasculitis is cutaneous vasculitis. It has been reported a female patient (26 years old) who was known with SLE, with red spots on both her legs and arms without feeling itchy or painful. Skin biopsy was done to the patient and the result was an appearance in skin tissue with the atrophic epidermis, hyperkeratosis, ketotic plague, thickening area of membrana basalis. In dermis underneath, was distributed inflammation cells perivascular, with lymphocyte and numerous PMN leukocyte. This appearance corresponds with discoid lupus erythematosus and vasculitis. Discoid lupus erythematosus is one most common cutaneous lupus erythematosus. In this case, the occurrence of vasculitis demonstrated an active disease of SLE. Disease activity was assessed with MEX SLEDAI and SLEDAI scores, which are 6 and 12 consecutively. The patient was given intravenous methylprednisolone 125 mg twice a day for 3 days and oral hydroxychloroquine 200 mg once daily. A good response was found in the sixth day with significant improvement of her skin lesions.Keywords: systemic lupus erythematosus, vasculitis

scholarly journals Ocular Complications in Cutaneous Lupus Erythematosus: A Systematic Review with a Meta-Analysis of Reported Cases

2015 ◽  
Vol 2015 ◽  
pp. 1-8 ◽  
Author(s):  
L. Arrico ◽  
A. Abbouda ◽  
I. Abicca ◽  
R. Malagola
Keyword(s):  
Lupus Erythematosus ◽  
Meta Analysis ◽  
Correct Diagnosis ◽  
Cutaneous Lupus Erythematosus ◽  
Discoid Lupus Erythematosus ◽  
Systemic Lupus ◽  
Ocular Complications ◽  
Ocular Symptoms ◽  
Inflammatory Syndrome ◽  
Cutaneous Lupus

Ocular complications associated with cutaneous lupus erythematosus (CLE) are less studied compared with those ones associated with systemic lupus erythematosus (SLE). The main ocular sites involved in patients affected by discoid lupus erythematosus (DLE) are eyelids followed by orbit and periorbit, the least being cornea. The most common complications are blepharitis usually affecting the lower lid and associated with some type of lid lesion such as plaque or erythematosus patches and madarosis. Few cases with LE profundus (LEP) and ocular complications are reported, but they are associated with orbital inflammatory syndrome and severe complications. The main treatment prescribed is hydroxychloroquine with a dose of 200 mg twice a day for 6 to 8 weeks. Corticosteroids are also used. Intervals between the correct diagnosis and the beginning of the ocular symptoms are commonly delayed. Ophthalmologist should be aware of the ocular manifestation of this autoimmune disease.

Expression pattern of tissue-resident memory T cells in cutaneous lupus erythematosus

Lupus ◽  
2021 ◽  
pp. 096120332110172
Author(s):  
Hyeon-Jung Gu ◽  
Shinyoung Song ◽  
Joo Young Roh ◽  
YunJae Jung ◽  
Hee Joo Kim
Keyword(s):  
T Cells ◽  
Lupus Erythematosus ◽  
Memory T Cells ◽  
Cutaneous Lupus Erythematosus ◽  
Discoid Lupus Erythematosus ◽  
Type I ◽  
Systemic Lupus ◽  
Peripheral Tissues ◽  
Resident Memory T Cells ◽  
Cutaneous Lupus

Background Tissue resident memory T cells (TRMs) persist long-term in peripheral tissues without recirculation, triggering an immediate protective inflammatory state upon the re-recognition of the antigen. Despite evidence incriminating the dysregulation of TRMs in autoimmune diseases, few studies have examined their expression in cutaneous lupus erythematosus (CLE). Objectives We aimed to examine whether there are differences among TRM populations in CLE depending on different clinical conditions, such as the CLE subtype or association with systemic lupus erythematosus, and to determine the effect of type I interferon (IFN) on the development of TRMs in CLE. Methods CLE disease activity was evaluated using the Cutaneous Lupus Erythematosus Disease Area and Severity Index. The expression of the TRM markers CD69 and CD103 in CLE lesions was evaluated by immunofluorescence. Flow cytometry was performed on peripheral blood mononuclear cells after IFNα treatment. Results The number of TRMs expressing either CD69 or CD103 was significantly higher in CLE lesions than in control skin; however, it was not significantly different between discoid lupus erythematosus and subacute CLE, or dependent on the presence of concomitant systemic lupus. Lesional severity was not correlated with an increase in TRMs in CLE. IFNα treatment induced a conspicuous increase in CD69 expression in skin-homing T cells, more profoundly in CD4+ T cells than in CD8+ T cells. Conclusions Skin TRMs, either CD69 or CD103-positive cells, showed increased levels in the lesional skin of CLE, and IFNα increased the expression of CD69 in T cells.

PD-1H (VISTA)–mediated suppression of autoimmunity in systemic and cutaneous lupus erythematosus

2019 ◽  
Vol 11 (522) ◽  
pp. eaax1159 ◽  
Author(s):  
Xue Han ◽  
Matthew D. Vesely ◽  
Wendy Yang ◽  
Miguel F. Sanmamed ◽  
Ti Badri ◽  
...  
Keyword(s):  
T Cells ◽  
Autoimmune Diseases ◽  
Lupus Erythematosus ◽  
Immune Cell ◽  
Cutaneous Lupus Erythematosus ◽  
Myeloid Cells ◽  
Discoid Lupus Erythematosus ◽  
Critical Element ◽  
Inhibitory Receptor ◽  
Cutaneous Lupus

Systemic lupus erythematosus (SLE) and discoid lupus erythematosus (DLE) of the skin are autoimmune diseases characterized by inappropriate immune responses against self-proteins; the key elements that determine disease pathogenesis and progression are largely unknown. Here, we show that mice lacking immune inhibitory receptor VISTA or programmed death-1 homolog (PD-1H KO) on a BALB/c background spontaneously develop cutaneous and systemic autoimmune diseases resembling human lupus. Cutaneous lupus lesions of PD-1H KO mice have clustering of plasmacytoid dendritic cells (pDCs) similar to human DLE. Using mass cytometry, we identified proinflammatory neutrophils as critical early immune infiltrating cells within cutaneous lupus lesions of PD-1H KO mice. We also found that PD-1H is highly expressed on immune cells in human SLE, DLE lesions, and cutaneous lesions of MRL/lpr mice. A PD-1H agonistic monoclonal antibody in MRL/lpr mice reduces cutaneous disease, autoantibodies, inflammatory cytokines, chemokines, and immune cell expansion. Furthermore, PD-1H on both T cells and myeloid cells including neutrophils and pDCs could transmit inhibitory signals, resulting in reduced activation and function, establishing PD-1H as an inhibitory receptor on T cells and myeloid cells. On the basis of these findings, we propose that PD-1H is a critical element in the pathogenesis and progression of lupus, and PD-1H activation could be effective for treatment of systemic and cutaneous lupus.

The combined role of clinical, reflectance confocal microscopy and dermoscopy applied to chronic discoid cutaneous lupus and subacutus lupus erythematosus: A case series and literature review

Lupus ◽  
2020 ◽  
Vol 30 (1) ◽  
pp. 125-133
Author(s):  
Sara Mazzilli ◽  
Laura Vollono ◽  
Laura Diluvio ◽  
Elisabetta Botti ◽  
Gaetana Costanza ◽  
...  
Keyword(s):  
Confocal Microscopy ◽  
Lupus Erythematosus ◽  
Cutaneous Lupus Erythematosus ◽  
Clinical Manifestations ◽  
Case Series ◽  
Skin Lesions ◽  
Reflectance Confocal Microscopy ◽  
Discoid Lupus Erythematosus ◽  
Cutaneous Lupus

Main subtypes of cutaneous lupus erythematosus are represented by acute, subacute cutaneous, intermittent and chronic cutaneous lupus erythematosus. Discoid lupus erythematosus represents the most common phenotype of chronic cutaneous lupus erythematosus. The spectrum of clinical manifestations mirrors that of several and distinct histopathological features. Such variability among different CLE subtypes is also observed at dermoscopy. Dermoscopy is nowadays considered an additional valuable method for skin lesions assessment in general dermatology, following and completing the well-known clinical diagnostic steps, such as medical history and clinical examination. In vivo reflectance confocal microscopy (RCM) is a non-invasive imaging tool able to assess the epidermis and upper dermis producing high resolution (horizontal ∼1.25 μm, vertical ∼5 μm), en face tissue sections used for melanocytic and inflammatory evaluation. In this study, we reported dermoscopic and RCM features about 9 patients affected by subacute and chronic lupus erythematosus retrospectively analyzed.

Spectrum of cutaneous lupus erythematosus in South Africans with systemic lupus erythematosus

Lupus ◽  
2019 ◽  
Vol 28 (8) ◽  
pp. 1021-1026 ◽  
Author(s):  
K Koch ◽  
M Tikly
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Renal Disease ◽  
Lupus Erythematosus ◽  
Odds Ratio ◽  
Systemic Disease ◽  
Cutaneous Lupus Erythematosus ◽  
Discoid Lupus Erythematosus ◽  
Systemic Lupus ◽  
South Africans ◽  
Cutaneous Lupus

Background Cutaneous involvement is very common in systemic lupus erythematosus. We describe the prevalence and spectrum of lupus-specific (cutaneous lupus erythematosus) and non-specific cutaneous features amongst mostly black South Africans with systemic lupus erythematosus. Patients and methods A retrospective record review of 298 South Africans (262 blacks and 36 non-blacks) with systemic lupus erythematosus was carried out. Cutaneous features were classified according to the Gilliam and Sontheimer classification of cutaneous lupus. Results Most (81.5%) patients were black African females. The mean (SD) age at diagnosis and follow-up duration were 35.0 (11.8) and 8.0 (5.9) years, respectively. Cutaneous lupus erythematosus was seen in 76.1% of patients, mainly chronic cutaneous lupus erythematosus with the discoid lupus erythematosus subtype seen in 52.1% of patients. Acute cutaneous lupus erythematosus was seen in 30.2% of patients and was more common in non-blacks than blacks (odds ratio = 3.8 (1.9–7.9)); localized acute cutaneous lupus erythematosus was more common than generalized acute cutaneous lupus erythematosus (odds ratio = 2.6 (1.6–4.4)). Non-specific cutaneous features occurred in 77.2%, with oral/nasal ulcers and Raynaud’s phenomenon each occurring in approximately 40% of patients. Diffuse melanonychia at initial diagnosis was present in 37.4% of patients and was more common in blacks than non-blacks (odds ratio = 3.1 (1.3–7.3)). Acute cutaneous lupus erythematosus was associated with renal disease (odds ratio = 2.8 (1.6–4.7)) and chronic cutaneous lupus erythematosus with arthritis (odds ratio = 2.02 (1.24–3.29)). Diffuse melanonychia was associated with less renal disease and anti-dsDNA antibody positivity (odds ratio = 0.4 (0.3–0.7) and 0.4 (0.2–0.6), respectively) and significantly lower lupus severity index scores (mean (SD) = 5.99 (1.11) vs 6.56 (1.36) in patients with no melanonychia, p < 0.05)). Conclusion In this study of South Africans with systemic lupus erythematosus, the skin was the most commonly affected organ. In general, cutaneous lupus erythematosus was associated with less severe systemic disease. Acute cutaneous lupus erythematosus was less common in blacks, whereas discoid lupus erythematosus was more common than reported in Caucasians. Diffuse melanonychia was a distinctive finding and was associated with milder systemic disease.

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