scholarly journals A Self-Limited Facial Rash in a Lupus Patient: The Case of Primary Facial Raynaud’s Phenomenon

2021 ◽  
pp. 366-371
Author(s):  
Kamel El-Reshaid ◽  
Shaikha Al-Bader ◽  
Hossameldin Tawfik Sallam
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Case Report ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Raynaud’S Phenomenon ◽  
Raynaud's Phenomenon ◽  
Joint Disease ◽  
Systemic Lupus ◽  
Malar Rash ◽  
Oral Ulcers

Skin is involved in 80% of systemic lupus erythematosus (SLE) and the second most affected after joint disease. Lupus-specific lesions include (a) acute ones viz. malar rash (80%), (b) subacute ones viz. photosensitive maculopapular dermatitis (50%), and (c) chronic ones viz. discoid rash. The lupus nonspecific lesions include; (a) nonscarring alopecia (86.67%), oral ulcers (56.67%), vasculitic lesions (33.34%), bullous lesions (10%), and Raynaud’s phenomenon (6.67%). In this case report, we describe a patient with SLE and antiphospholipid antibodies that had developed a transient facial form of Raynaud’s phenomenon that was not associated with disease activity and digital changes. Its association with SLE is discussed.

Secondary Sjögren’s Syndrome in Systemic Lupus Erythematosus Defines a Distinct Disease Subset

2010 ◽  
Vol 37 (6) ◽  
pp. 1143-1149 ◽  
Author(s):  
ALAN N. BAER ◽  
JANET W. MAYNARD ◽  
FASIL SHAIKH ◽  
LAURENCE S. MAGDER ◽  
MICHELLE PETRI
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Renal Disease ◽  
Lupus Erythematosus ◽  
Raynaud’S Phenomenon ◽  
Raynaud's Phenomenon ◽  
Sjogren's Syndrome ◽  
Systemic Lupus ◽  
Oral Ulcers ◽  
Laboratory Features ◽  
Dsdna Antibodies

Objective.Sjögren’s syndrome (SS) may occur in patients with systemic lupus erythematosus (SLE). We sought to determine whether the presence of SS in a large cohort of patients with SLE defines a subset with distinctive sociodemographic, clinical, and laboratory features.Methods.The Johns Hopkins Lupus Cohort was divided into 2 groups, based on the presence or absence of SS, defined by the presence of an objective measure of sicca or an abnormal minor salivary gland biopsy in a patient with sicca symptoms. These groups were compared with regard to sociodemographic, clinical, and laboratory features. Multivariable logistic regression was then performed to adjust the findings for potential sociodemographic, clinical, and laboratory confounders.Results.The 259 patients with SS (14% of the cohort), when compared with the 1531 patients without SS, were older at the time of SLE diagnosis and were more commonly women and white. Photosensitivity, oral ulcers, Raynaud’s phenomenon, anti-Ro antibodies, and anti-La antibodies had a significant positive association while renal disease, anti-ribonucleoprotein (RNP) antibodies, and anti-dsDNA antibodies had a negative association with the presence of SS after adjustment for age (at last cohort visit), gender, ethnicity, and anti-Ro antibodies. The older age at diagnosis of SLE among the patients with SS did not remain a significant finding after adjustment for the age of the patient at last cohort visit.Conclusion.The subset of patients with SLE and SS has a distinct clinical and laboratory phenotype, with a higher frequency of older white women with photosensitivity, oral ulcers, Raynaud’s phenomenon, anti-Ro antibodies, and anti-La antibodies and a lower frequency of renal disease, anti-dsDNA antibodies, and anti-RNP antibodies.

SAT0217 PERFORMANCE OF ACR/EULAR 2019, SLICC 2012 AND ACR 1997 CLASSIFICATION CRITERIA IN A COHORT OF SYSTEMIC LUPUS ERYTHEMATOSUS PATIENTS WITH LONGSTANDING DISEASE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1051-1052
Author(s):  
D. Lobo Prat ◽  
B. Magallares ◽  
I. Castellví ◽  
H. Park ◽  
P. Moya ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Statistical Significance ◽  
Renal Involvement ◽  
High Sensitivity ◽  
Classification Criteria ◽  
Average Score ◽  
Systemic Lupus ◽  
Malar Rash ◽  
Oral Ulcers

Background:Systemic lupus erythematosus (SLE) is an autoimmune disease with variable clinical features and a complex physiopathology. In 2019, EULAR and ACR have jointly developed new classification criteria with both high sensitivity and specificity. These criteria have the particularity of including the presence of ANA as an obligatory entry criterion and the existence of clinical and immunological domains with weighted scores.Objectives:To evaluate the performance and characteristics of the ACR/EULAR 2019, SLICC 2012 and ACR 1997 classification criteria in a cohort of SLE patients with longstanding disease.Methods:Descriptive observational study that enrolled a cohort of SLE patients with longstanding disease followed in a tertiary level hospital. Demographic and clinical data were gathered along with the fulfillment of classification criteria. The sensitivity of each classification criteria and the statistically significant associations between criteria fulfillment and clinical and immunological data were calculated. Statistical analyses were performed using the Chi2, T-student and ANOVA tests. Statistical significance was assumed in p values <0.05.Results:A total of 79 patients (88.6% women) with a mean age of 51.8±14 years, disease duration of 15.2±11.5 years and SLEDAI of 2.65±2.1 were included. The sensitivity of the different classification criteria was 51.9% for ACR 1997, 87.3% for SLICC 2012 and 86.1% for ACR/EULAR 2019 (Table 1).Table 1.Sensitivity and average scores.ACR/EULAR 2019SLICC 2012ACR 1997Sensitivity (%)86.187.351.9Average score of patients classified as SLE(±SD)18.6±5.85.3±1.45±0.9Average score of patients NOT classified as SLE(±SD)6.1±2.52.8±0.42.8±0.851.9% of patients met all three classification criteria, 29.1% met SLICC 2012 and ACR/EULAR 2019, 5% only met SLICC 2012 and 3.7% exclusively met ACR/EULAR 2019. 11.4% of patients did not meet any classification criteria and were characterized by having a low SLEDAI (0.6±0.9) and fulfilling only skin domains (alopecia or oral ulcers), antiphospholipid antibodies or hypocomplementemia.Statistically significant associations were found between meeting ACR/EULAR 2019 classification criteria and the presence of low C3 and C4 (p<0.04), DNA (p<0.001), lupus nephritis III-IV (p<0.05) and arthritis (p<0.001), highlighting that all patients with arthritis met these criteria.In the SLICC 2012 evaluation, significant associations were found between meeting these criteria and the presence of arthritis (p<0.01), renal involvement (p<0.04), leukopenia/lymphopenia (p=0.05), DNA (p<0.03) and hypocomplementemia (p=0.02).Fullfilment of ACR 1997 was associated to the presence of malar rash (p<0.001), discoid lupus (p<0.05), photosensitivity (p<0.001) and oral ulcers (p<0.04), as well as arthritis (p<0.001), serositis (p=0.02), renal (p<0.05) and hematologic (p=0.05) involvement.The Kappa concordance coefficient among classification criteria is detailed in Table 2.Table 2.Kappa concordance coefficient.ACR/EULAR 2019 - SLICC 2012ACR/EULAR 2019 - ACR 1997SLICC 2012 - ACR 1997Kappa concordance coefficient0.610.270.30Conclusion:The ACR/EULAR 2019 classification criteria maintain a high sensitivity similar to the SLICC 2012 in SLE patients with longstanding disease, both of which are much higher than ACR 1997. Patients with serological, articular or renal involvement are more likely to meet SLICC 2012 or ACR/EULAR 2019 criteria. It is noteworthy the relevance of dermatological manifestations in ACR1997 classification criteria against the increased weight that a better understanding of SLE physiopathology has provided to analytic and immunological criteria in the subsequent classification criteria.Disclosure of Interests:David Lobo Prat: None declared, Berta Magallares: None declared, Ivan Castellví Consultant of: Boehringer Ingelheim, Actelion, Kern Pharma, Speakers bureau: Boehringer Ingelheim, Actelion, Bristol-Myers Squibb, Roche, HyeSang Park: None declared, Patricia Moya: None declared, Ignasi Gich: None declared, Ana Laiz: None declared, Cesar Díaz-Torné: None declared, Ana Milena Millán Arciniegas: None declared, Susana P. Fernandez-Sanchez: None declared, Hector Corominas: None declared

Systemic sclerosis

2010 ◽  
pp. 3664-3678
Author(s):  
Christopher P. Denton ◽  
Carol M. Black
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Systemic Sclerosis ◽  
Lupus Erythematosus ◽  
Raynaud’S Phenomenon ◽  
Raynaud's Phenomenon ◽  
Systemic Lupus ◽  
Overlap Syndromes ◽  
Autoimmune Rheumatic Disease ◽  
Skin Sclerosis ◽  
Important Form

The scleroderma spectrum of disorders includes a number of diseases that have Raynaud’s phenomenon or skin sclerosis in common, comprising (1) limited cutaneous scleroderma; (2) systemic sclerosis (SSc)—the most important form of scleroderma—limited cutaneous SSc, diffuse cutaneous SSc, and overlap syndromes (with features of another autoimmune rheumatic disease, e.g. systemic lupus erythematosus); (3) Raynaud’s phenomenon—autoimmune (with antinuclear or other SSc-associated antibodies) or primary. These conditions affect women four times as often as men, most often beginning in the fifth decade....

Is there a difference in systemic lupus erythematosus with and without Raynaud’s phenomenon?

2012 ◽  
Vol 33 (4) ◽  
pp. 859-865 ◽  
Author(s):  
Slavica Pavlov-Dolijanovic ◽  
Nemanja S. Damjanov ◽  
Nada Z. Vujasinovic Stupar ◽  
Danijel R. Marcetic ◽  
Mirjana N. Sefik-Bukilica ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Raynaud’S Phenomenon ◽  
Raynaud's Phenomenon ◽  
Systemic Lupus

Electroacupuncture Therapy for Arthralgia and Raynaud's Phenomenon in a Patient with Systemic Lupus Erythematosus

2010 ◽  
Vol 28 (1) ◽  
pp. 49-51 ◽  
Author(s):  
Nozomi Donoyama ◽  
Norio Ohkoshi
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Visual Analogue Scale ◽  
Lupus Erythematosus ◽  
Analogue Scale ◽  
Raynaud’S Phenomenon ◽  
Raynaud's Phenomenon ◽  
Systemic Lupus ◽  
Cold Provocation ◽  
Temperature Recovery ◽  
The Right

A 45-year-old woman with systemic lupus erythematosus presented with multiple arthralgia, coldness in fingers and toes, and Raynaud's phenomenon. Electroacupuncture (EA) therapy was performed in two courses (14 treatment sessions) 1 month apart. A needle was inserted in the proximal (or medial) side of the painful joint and another needle was inserted in the distal (or lateral) side of the same joint and a 50 Hz stimulus was applied (3 s bursts with 1 s gaps) for 15 min. A visual analogue scale was used to evaluate pain intensity. Cold provocation testing was conducted before and after EA sessions to determine the vasomotor response. Visual analogue scale scores were lower after EA sessions than before. Before starting EA, the skin temperature of the right mid fingertip was 27.9°C and that of the left mid fingertip was 28.3°C. In contrast, after the EA sessions, the skin temperature of the right mid fingertip was 34.8°C and that of the left mid fingertip was 34.7°C. In the last EA session, the patient reported that the cold in her fingers and toes had eased and Raynaud's phenomenon, in which nail colour tone changed from white to red, had disappeared. In the cold-provocation test, before EA, the temperature recovery rates of mid fingertips after cold exposure reached over 80% in 20 min. In contrast, after EA had been completed, the temperature recovery rate exceeded 80% in 10 min, thus the delay of temperature recovery was alleviated.

scholarly journals Immune Thrombocytopenic Purpura as Initial Presentation of Paediatric SLE: A Case Report

2020 ◽  
Vol 59 (233) ◽  
Author(s):  
Anjila Ghimire
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Platelet Count ◽  
Case Report ◽  
Lupus Erythematosus ◽  
Female Child ◽  
Connective Tissue Disorder ◽  
Systemic Lupus ◽  
Thrombocytopenic Purpura ◽  
Malar Rash ◽  
Mucosal Bleeding

The systemic lupus erythematosus (SLE) is a connective tissue disorder with variable presentationsin children. The usual presentation includes arthritis, malar rash, nephritis, hemolytic anemia, andfever. Isolated hematologic abnormality as the only presentation of SLE is rare. Here is a case reportof a female child presented to us with superficial and mucosal bleeding with isolated low plateletcount and anemia in proportion to blood loss. When platelet count didn’t go up despite appropriatetreatment in lines of ITP, further investigations were done, diagnosis of SLE was established, andmanagement was done accordingly.

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