Diagnostic algorithm for Raynaud’s phenomenon and vascular skin lesions in systemic lupus erythematosus

Lupus ◽  
2010 ◽  
Vol 19 (9) ◽  
pp. 1087-1095 ◽  
Author(s):  
JG Richter ◽  
O. Sander ◽  
M. Schneider ◽  
P. Klein-Weigel
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Clinical Features ◽  
Lupus Erythematosus ◽  
Raynaud’S Phenomenon ◽  
Correct Diagnosis ◽  
Clinical Manifestations ◽  
Diagnostic Algorithm ◽  
Skin Lesions ◽  
Raynaud's Phenomenon ◽  
Systemic Lupus

Skin discolorations and skin lesions due to vascular pathologies are common clinical features in systemic lupus erythematosus. A variety of clinical manifestations such as Raynaud’s phenomenon, acrocyanosis, livedo patterns, erythematous or violaceous macules and papules or necrosis are triggered by heterogeneous pathophysiological mechanisms such as vasospasm, vasculitis or thromboembolism. A standardized macro- and microvascular assessment is necessary to establish the correct diagnosis. We describe and illustrate common clinical features of vascular skin manifestations in systemic lupus erythematosus and present a diagnostic algorithm. Lupus (2010) 19, 1087—1095.

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....

Overlap/undifferentiated syndromes

2013 ◽  
pp. 1069-1078 ◽  
Author(s):  
Ariane Herrick
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Clinical Features ◽  
Lupus Erythematosus ◽  
Raynaud’S Phenomenon ◽  
Clinical Manifestations ◽  
Raynaud's Phenomenon ◽  
Overlap Syndromes ◽  
Clear Cut ◽  
Positive Treatment

Undifferentiated connective tissue disease (UCTD) and overlap syndromes both form part of the broad spectrum of connective tissue disease. They are difficult to define, as the boundaries between them and specific diseases such as systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and myositis are often not clear-cut. This chapter gives a broad overview of diagnosis, clinical features, outcomes, and management. Patients with UCTD have clinical and/or serological features of connective tissue disease but do not fulfil the criteria for any one defined disease. Raynaud’s phenomenon and puffy fingers are often the presenting features but there are many possible others, including arthralgia, sicca symptoms, and breathlessness due to pulmonary fibrosis, usually in the context of a positive anti-nuclear antibody (ANA). A proportion of patients evolve into a defined connective tissue disease: in those who do, this is generally within 5 years of onset. Treatment is dependent upon the clinical features: for examplem vasodilators for Raynaud’s phenomenon, or hydroxychloroquine for arthralgia/arthritis. Patients with overlap syndromes have features of more than one defined connective tissue disease. Overlap syndromes are therefore highly heterogeneous as many combinations of clinical and serological features can occur. Mixed connective tissue disease (MCTD) is the overlap syndrome that has been most described and includes overlapping features of SLE, SSc, and myositis in patients who are anti-U1 ribonucleoprotein (RNP) antibody positive. Treatment is of the specific clinical manifestations. Patients with overlap syndromes should be kept under regular review to allow early identification of internal organ involvement.

Systemic lupus erythematosus—clinical features and aetiopathogenesis

2013 ◽  
pp. 923-937
Author(s):  
Caroline Gordon
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Differential Diagnosis ◽  
Autoimmune Disease ◽  
Clinical Features ◽  
Lupus Erythematosus ◽  
Immune Complexes ◽  
Clinical Manifestations ◽  
Systemic Lupus ◽  
Life Threatening ◽  
Symptoms And Signs

Systemic lupus erythematosus (SLE or lupus) is a multisystem, autoimmune disease associated with the formation of autoantibodies that form pathological immune complexes and activate a number of inflammatory pathways. The disease is characterized by remissions and relapses (flares) that can present with a variety of clinical manifestations. The symptoms and signs may range from mild features that can be treated easily to organ and even life threatening manifestations requiring potent immunosuppression. This chapter will review the epidemiology and pathology of lupus, then the clinical features including differential diagnosis and investigation of adult patients with SLE. Finally the classification, diagnosis, monitoring and outcome of lupus patients will be discussed.

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

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.

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 Pulmonary Hypertension in Systemic Lupus Erythematosus with Raynaud’s Phenomenon: Case Report

2021 ◽  
Vol 13 (1) ◽  
pp. 476-482
Author(s):  
Agnes Dina Irene Dorithy Zagoto ◽  
Ayu Paramaiswari
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Pulmonary Hypertension ◽  
Lupus Erythematosus ◽  
Rare Complication ◽  
Raynaud’S Phenomenon ◽  
Signs And Symptoms ◽  
Right Heart Failure ◽  
Raynaud's Phenomenon ◽  
Systemic Lupus ◽  
Multiple Organs

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects multiple organs such as skin, joints, kidneys, heart, lungs, nervous system and blood. Pulmonary hypertension is a rare complication of SLE.1 Systemic lupus erythematosus associated with pulmonary hypertension was defined as an increase in the average pressure in the pulmonary artery at rest ? 25 mmHg with pulmonary capillary wedge pressure ?15 mm Hg and an increase in pulmonary vascular resistance.2 The prevalence of pulmonary hypertension in SLE approximately 0.5 to 17.5%. Predictors factors of the occurrence of pulmonary hypertension in LES is Raynaud's phenomenon, anti-U1RNP antibody, and antibody positive anticardiolipin.3 A woman aged 37 years came with a chief complaint of pain in the fingers and toes with black-colored wounds felt since 6 months before admission. From the anamnesis, physical examination, support to meet 5 of the classification criteria for systemic lupus erythematosus based on the 1997 ACR criteria which includes manifestations of arthritis, mucocutaneous, serositis, lupus antiokoagulan, and ANA IF positive. In these patients also found the typical signs and symptoms of Raynaud's phenomenon which leads to the symptoms of pain in the fingers of both hands when exposed to cold and pale to red when heated and has been confirmed from the results of arteriography. From the results of echocardiography reveal any pulmonary hypertension. This patient was treated with steroids, immunosuppressants and antiplatelet. The case was removed because of pulmonary hypertension is a complication LES rare and necessary sharpness in diagnosis. Patients with pulmonary hypertension of unknown or untreated can become a progressive right heart failure and lead to death.3

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