Your doctor may also prescribe one or more of these types of drugs:
There is not enough evidence to recommend most natural remedies for treating lupus. If you want to incorporate natural therapies into your care, be sure to talk about it with your healthcare provider first. They can help you determine which therapies might complement your treatment plan best and how to choose the right products.
Saphnelo approved in the EU for the treatment of moderate to severe systemic lupus erythematosus
Corticosteroids. Prednisone and other types of corticosteroids can counter the inflammation of lupus. High doses of steroids such as methylprednisolone (Medrol) are often used to control serious disease that involves the kidneys and brain.
Early Progress With Lupus Treatment Involves Antimalarials and Corticosteroids. The first medication used for lupus, quinine (an antimalarial), was described by Payne in 1894 (Payne, 1894).
Prednisone is the most common steroid that doctors use to treat lupus. If you have liver problems, your doctor may recommend different steroids called prednisolone or methylprednisolone (Medrol®). There are a few different ways to take steroids: Most people take steroids as pills.
Lupus is a chronic autoimmune disease in which the immune system mistakenly attacks healthy body cells and tissues. Doctors may use chemotherapy drugs to treat lupus. These medications suppress overactive immune reactions, helping ease inflammation, limit organ damage, and improve quality of life.
Systemic lupus erythematosus (SLE), commonly known as lupus, is an autoimmune illness that affects all parts of the body. The term "lupus" is also...
The exact cause of lupus is unknown, but most scientists believe that genetics, combined with outside triggers – such as infections, medications or...
There are five recognized forms of lupus: Systemic lupus erythematosus (SLE) is the disease most commonly mentioned, and the most serious since it...
Gender, race and ethnicity, and age are all key factors. Younger women, and especially younger women of color, are most at risk.
People with rheumatic diseases and suppressed immune systems, such as lupus patients, may be more vulnerable to the disease known as COVID-19, whic...
Symptoms vary from person to person, but the typical lupus patient is a young woman who develops arthritis of the fingers, wrists or other small jo...
A diagnosis for lupus is generally based on laboratory tests that exclude other diseases which may have similar symptoms (such as Lyme disease), an...
Depending on the symptoms, blood test results and the particular organs involved, a person with lupus may receive one or more of the following: non...
The severity of lupus varies from mild to life-threatening. After many years of having lupus, patients may develop: osteoporosis (especially in tho...
Lupus patients who are positive for antiphospholipid antibody (aPL) can develop blood clots and heart valve disease and may require additional medi...
Connect with others who have lupus. Talk to other people who have lupus. You can connect through support groups in your community or through online message boards. Other people with lupus can offer unique support because they're facing many of the same obstacles and frustrations that you're facing.
Laboratory tests. Blood and urine tests may include: Complete blood count. This test measures the number of red blood cells, white blood cells and platelets as well as the amount of hemoglobin, a protein in red blood cells. Results may indicate you have anemia, which commonly occurs in lupus. A low white blood cell or platelet count may occur in ...
Erythrocyte sedimentation rate. This blood test determines the rate at which red blood cells settle to the bottom of a tube in an hour.
Examples include azathioprine (Imuran, Azasan), mycophenolate (Cellcept), methotrexate (Trexall, Xatmep, others), cyclosporine (Sandimmune, Neoral, Gengraf) and leflunomide (Arava). Potential side effects may include an increased risk of infection, liver damage, decreased fertility and an increased risk of cancer.
Side effects include weight gain, easy bruising, thinning bones, high blood pressure, diabetes and increased risk of infection. The risk of side effects increases with higher doses and longer term therapy. Immunosuppressants. Drugs that suppress the immune system may be helpful in serious cases of lupus.
Signs and symptoms of lupus may change over time and overlap with those of many other disorders. No one test can diagnose lupus. The combination of blood and urine tests, signs and symptoms, and physical examination findings leads to the diagnosis.
Corticosteroids. Prednisone and other types of corticosteroids can counter the inflammation of lupus. High doses of steroids such as methylprednisolone (Medrol) are often used to control serious disease that involves the kidneys and brain. Side effects include weight gain, easy bruising, thinning bones, high blood pressure, diabetes and increased risk of infection. The risk of side effects increases with higher doses and longer term therapy.
In particular, hydroxychloroquine is associated with a higher rate of remission, fewer relapses, and reduced damage in the course of the disease, even in lupus nephritis. High-dose glucocorticoids should be given only when acutely indicated; immunosuppressives such as azathioprine, methotrexate, or mycophenolate mofetil may be administered to reduce glucocorticoids, according to the EULAR recommendations. Belimumab was recently approved as add-on therapy in autoantibody-positive SLE patients with high disease activity unresponsive to standard treatment. Short-term induction pulse therapy with low-dose intravenous cyclophosphamide, as well as continued mycophenolate mofetil treatment are advances in lupus nephritis.
For classification as SLE, four criteri a (at least one of them clinical and at least one immunological) have to be fulfilled or lupus nephritis has to be diagnosed histologically in the presence of ANA or anti-dsDNA antibodies. The SLICC criteria are not diagnostic criteria.
Cutaneous manifestations occur in about 75% of patients with SLE in the course of the disease, and are the first sign in a quarter of cases (e10). Based on clinical and histological criteria, the skin lesions are divided into lupus erythematosus (LE)-specific (eTable) and LE-non-specific manifestations (e11, e12). The most frequent LE-specific manifestation is the acute cutaneous lupus erythematosus (ACLE), which may occur as a butterfly rash or in the form of a generalized maculopapular exanthema. Discoid lupus erythematosus (DLE) displays a chronic course, typically characterized by inflammatory erythematous plaques with follicular hyperkeratosis and scarring (Figure 1a), leading to irreversible alopecia in hairy areas (Figure 1b). Approximately 5% of patients with DLE who initially show no systemic organ involvement will develop SLE. Subacute cutaneous lupus erythematosus (SCLE) is characterized by symmetric, annular, polycyclic, and/or papulosquamous/psoriasiform skin lesions without scarring on sun-exposed areas of the back, chest, and extensor surfaces of the arms (Figure 1c). The LE-non-specific manifestations include, among others, vascular skin lesions (e.g., periungual teleangiectasia, livedo racemosa, Raynaud syndrome).
Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease that may involve many different organs and display a variable clinical course. The diagnosis of SLE is based on characteristic clinical findings of the skin, joints, kidneys, and the central nervous system, as well as on serological parameters such as antinuclear antibodies (ANA), in particular antibodies to dsDNA (e1). The various clinical symptoms do not always occur simultaneously and may develop at any stage of the disease. In the early stages, physicians from various disciplines often propose several differential diagnoses, or identify only one aspect of the disease without recognizing the symptoms as part of SLE (1, e2). Fever, fatigue, and arthralgia are the most frequently occurring non-specific symptoms at disease onset; additional joint swelling or a "butterfly rash"”particularly in women of childbearing age”should prompt consideration of SLE (2). The aim of this article is to provide an updated review on the diagnosis and treatment of SLE, based on a selective survey of the literature in PubMed and the Cochrane Library, including current guidelines and the recommendations of experts with extensive experience in the management of this disease.
Fever, fatigue, and arthralgia are the most frequently occurring non-specific symptoms at disease onset; additional joint swelling or a "butterfly rash"”particularly in women of childbearing age”should prompt consideration of SLE (2).
Hydroxychloroquine and chloroquine are licensed for the treatment of SLE. Apart from their good efficacy against arthritis and LE-specific skin lesions (8), antimalarials maintain SLE in remission, are associated with fewer disease flares, and reduce damage in the course of the disease (23, e18).
What is lupus? Systemic lupus erythematosus (SLE), commonly known as lupus, is an autoimmune illness that affects many organs and systems in the body. Lupus is a chronic condition, but symptoms tend to cycle in alternate periods of "flares" (or "flares-ups") and remissions.
If your primary care doctor suspects you may have lupus, contact a rheumatologist to confirm the diagnosis and get appropriate treatment. HSS is top-ranked for rheumatology by U.S. News & World Report and offers a dedicated team of specialists and resources at the Lupus and APS Center of Excellence.
The incidences of lupus in women of African descent is three times that of incidences in Caucasian women. The incidences of lupus in women of Asian descent are twice that of incidences in Caucasian women. The incidences of lupus in women of Latin descent are twice that of incidences in Caucasian women. Age: Symptoms that lead to a lupus diagnosis ...
SLE, commonly just called lupus, is an autoimmune disease that affects as many as 500,000 people in the United States. In people with the condition, the immune system malfunctions and, instead of attacking infections like it’s supposed to, it attacks healthy tissues. The organs and systems, most commonly affected by lupus include the skin, joints, kidneys and lungs.
Symptoms vary from person to person, but the typical lupus patient is a young woman who develops arthritis of the fingers, wrists or other small joints, hair loss, a rash (often on the face, in butterfly pattern over the nose and cheeks).
Age: Symptoms that lead to a lupus diagnosis most commonly appear in people between 15 and 44 years of age.
Gender, race and ethnicity, and age are all key factors. Younger women, and especially younger women of color, are most at risk. Gender: Most lupus patients are female. The ratio of women to men who have lupus is about 9 to 1.
Patients suffering from SLE are typically treated with corticosteroids and immunosuppressive agents (1). An eminent direct or indirect target of novel therapeutic approaches has been the lupus B cell (2–4). Among them, only belimumab that inhibits B cell survival has been approved for patients with SLE and SLE-related nephritis. Rituximab (RTX) causing B cell depletion can also be administered according to the ACR and EULAR guidelines in refractory lupus nephritis despite failed clinical trials, and is often used off-label for other manifestations as well, based on the encouraging results of diverse studies. This reflects one of the problems of failed clinical trials in patients with SLE: failure to suppress one specific SLE manifestation, such as lupus nephritis, may not exclude encouraging outcomes for some other aspects of the disease, such as hematological, mucocutaneous, or articular involvement. Inadequate control of lupus nephritis may potentially result to end-stage renal disease due to irreversible damage of the kidneys. Measurement of proteinuria is a useful tool to assess disease activity in patients with kidney involvement and an early renal response is judged by a decrease of proteinuria; improvement of proteinuria at 12 months of treatment correlates well with a favorable long-term renal outcome. Despite progress, a complete renal response is not achieved in more than 40% of patients with lupus nephritis. Other manifestations are also commonly less-than-satisfactorily treated. Therefore, additional and new approaches are being evaluated.
B cells are being targeted directly or indirectly in patients with lupus. RC18 is a recombinant human BLyS receptor antibody fusion protein and it is used in a phase III placebo-controlled study plus standard treatment with primary outcome an SRI response rate at week 52 (59).
Daratumumab, a mAb approved for the treatment of multiple myeloma, is an IgG1k mAb directed against CD38 causing depletion of plasma cells. Long-lived plasma cells are residents in niches in the bone marrow or (perhaps more importantly) in inflamed tissue and they do not respond to immunosuppressants, including B-cell-targeting treatments. Two patients with severe manifestations of SLE received daratumumab at a dose of 16 mg/kg of body weight once a week for 4 weeks followed by maintenance treatment with I.V. belimumab ( 18 ). Daratumumab treatment resulted in remarkable clinical outcomes not only of severe manifestations such as lupus nephritis, autoimmune hemolytic anemia and autoimmune thrombocytopenia but also on less severe manifestations such as arthritis, skin rashes, pericarditis, cutaneous vasculitis, alopecia, and mucosal ulcers. Daratumumab treatment was also associated with favorable serologic responses. Importantly, previous therapeutic interventions with a variety of agents such as bortezomib, mycophenolate mofetil, and cyclophosphamide were ineffective. Despite the extremely small number of patients, data are encouraging supporting further evaluation of daratumumab in meaningfully larger numbers of patients with SLE. It is of interest however that the authors did not ascribe their anti-CD38 mAb-mediated clinical effect (s) exclusively to reductions of plasma cell numbers. Other circulating cells also express CD38 and their numbers decreased following daratumumab treatment. Among them are subsets of B cells, plasmacytoid dendritic cells, and a greatly expanded CD38 + T cell subpopulation. Only recently it was shown by Katsuyama et al. that this expanded CD38 + CD8 + T cell subset is responsible for the significantly compromised cytotoxicity encountered in patients with lupus ( 19 ).
Lulizumab is a mAb against CD28, the T cell costimulatory molecule that is essential for T cell activation. In a phase II 24-week study, lulizumab was administered at a dose of 12.5 mg/week or at doses of 1.25 and 5 mg, 12.5 every other week or placebo in combination with standard treatment in 349 patients with SLE (16). Measurement tools of disease activity such as the British Isles Lupus Assessment Group Based Composite Lupus Assessment (BICLA) response rate, CLASI (Cutaneous Lupus Erythematosus Disease Area and Severity Index), and SLEDAI (Systemic Lupus Erythematosus Disease Activity Index) did not show any significant changes between groups.
To formally address the question of its efficacy and safety in lupus nephritis, an international phase III, 104-week , randomized, double-blind, placebo-controlled trial of intravenous (IV) belimumab (BLISS-LN) in addition to standard treatment was recently completed (28). A total of 448 patients were randomized to receive belimumab or placebo (1:1). The primary end point was the primary efficacy renal response at week 104, an endpoint that excluded partial renal response and was defined as an urinary protein to creatinine ratio (UPCR) of 0.7 or less, an estimated glomerular filtration rate (eGFR) that had not declined more than 20% below the levels before the flare or was >60 ml/min/1.73 m2and no use of rescue therapy in cases of treatment failure. Primary efficacy renal response was noticed in 43% of the patients that were treated with belimumab given on top of standard treatment and in 32% of the patients that were treated with placebo in combination with standard treatment (p= 0.03) at week 104. Complete renal response at week 104 was one of the major secondary end points and was defined as an UPCR of <0.5, an eGFR that did not decline more than 10% below the levels before the flare, or was >90 ml/min/ 1.73 m2and no use of rescue treatment in cases of therapy failure. More patients in the belimumab group compared to the placebo group had a complete renal response at week 104 (30 vs. 20%; p= 0.02). The risk of death or a renal-associated event was also a secondary end point and was significantly lower in the belimumab group compared to the placebo group (HR: 0.51, p= 0.001). Regarding safety, no differences were recorded between the two groups of patients. Consequently, the addition of belimumab on top of standard of care may work better in patients with lupus nephritis without particular concerns regarding safety. Although a significant number of patients with lupus nephritis was enrolled in each arm of the study, no subgroups of the patients that might benefit the most from belimumab treatment were identified. In addition, although a better outcome was recorded in 11% more patients, the percentages of responding patients are still far from impressive. The FDA recently approved intravenous belimumab for the treatment of patients with lupus nephritis.
The B cell, as a major component of the adaptive immune system, may mediate autoimmune disease. B cells are not only capable of producing autoantibodies after their differentiation into plasma cells, but they also present autoantigens to T cells and they secrete cytokines. Therefore, B cells represent an established and clear target of treatment approaches; lupus B cells have been targeted either directly via regimens that cause B cell depletion or indirectly via regimens affecting B cell survival, or via inhibiting their antigen-receptor-initiated function.
The B cell has been targeted in SLE since decades. Initially considered guilty only as autoAb producers, B cells were subsequently also recognized as efficient antigen-presenting cells and cytokine producers. Works from the Craft Lab disclosed that murine lupus could indeed develop in T cell deficient animals ( 5 ). In contrast, it was principally with the works of Chan et al. that a central, eminent, and indispensable pathogenetic role was assigned to the B cell in murine lupus models ( 6, 7 ). In humans, critical functions of the B cell, such as the antigen-receptor initiated activation was revealed to be intrinsically abnormal (Liossis et al., work from the Tsokos Lab) ( 2 ). Anolik and Leandro from the Departments of Looney and Isenberg, respectively, were the first to administer the B cell depleting mAb RTX in a few patients with SLE with promising results ( 8, 9 ).
Localized disease is initially treated with topical agents (either corticosteroids (CS) or calcineurin inhibitors (CI)). Hydroxychloroquine (HCQ) is also often used, depending on the site or if there is scarring disease. Widespread or scarring disease treatment starts with topicals and HCQ. If this fails, quinacrine is added to HCQ. If this regimen fails, a switch to chloroquine (CQ) can be made, while continuing quinacrine. If this fails, other options include mycophenolate mofetil (MM) or mycophenolate sodium (MS), azathioprine, dapsone, retinoids and thalidomide can be considered. In the case of failure of these agents, experimental therapy can be considered.
Cutaneous lupus erythematosus comprises a range of dermatologic manifestations, including acute cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus. The ACR criteria, which include four cutaneous signs, may lead to overdiagnosis of SLE in patients with predominantly cutaneous disease. Diagnosis of CLE requires proper classification of the subtype, which is best accomplished by a focus on the clinical and histologic findings. Serology and direct immunofluorescence are less helpful in making the diagnosis. CLE treatment combines sun protection, topical therapies, and systemic agents. Antimalarials are considered first line treatment. Multiple agents are under investigation as alternative therapies.
Lupus tumidus is a subtype of CCLE characterized by extreme photosensitivity and a benign course occurring preferentially in men. Clinically, these lesions appear on the face as erythematous, edematous, urticaria-like polycylic plaques with sharp raised borders and smooth surfaces. Unlike classic DLE lesions, follicular plugging does not occur. Histologically, these lesions exhibit a dense perivascular and periadnexal infiltrate without involvement of the interface. DIF testing is typically negative, and 10% of patients are ANA positive [35]. Some authors have suggested a separate category for LET, entitled Intermittent Cutaneous Lupus Erythematosus (ICLE), but there is not agreement and there are some who feel this could also be a lupus-associated skin disease [22].
Chilblain Lupus (CHLE) is a rare form of CCLE resembling frostbite. Lesions appear as painful, violaceous plaques and nodules in cold-exposed areas. Central erosions or ulcerations may occur on acral surfaces, such as fingers, toes, heels, nose, and ears. Chilblain lupus occurs when there is a temperature drop, and can be difficult to distinguish from frostbite. Pathology shows epidermal atrophy, interface vacuolization, and a perivascular mononuclear infiltrate. Twenty percent of patients with CHLE develop features of SLE at some point in their disease course [34].
The cornerstone of CLE diagnosis is a lesional biopsy for histology. Histologic findings vary by subtype, but in general CLE lesions share the features of vacuolar or hydropic change and lymphocytic infiltrates. Direct immunofluorescence (DIF) of lesional biopsies can supplement non-definitive histologic findings. The lesional lupus band test refers to the finding of immunoglobulins and complement at the dermal-epidermal junction of a lesional biopsy, a classic finding in CLE. Deposits are typically granular in appearance, and most commonly contain IgG and IgM, although IgA can be found [47]. Although CLE lesions generally have a positive lesional lupus band test, a negative test does not exclude the diagnosis. Likewise, a positive lesional lupus band test does not secure the diagnosis, as false positive tests can occur in sun-damaged skin. In most cases, clinical and histologic findings provide sufficient information to make a diagnosis of CLE, and therefore a DIF is usually unnecessary. Non-lesional lupus band tests are seen in SLE, and have been reported in multiple other autoimmune diseases, including rheumatoid arthritis, Sjogren’s syndrome, dermatomyositis, scleroderma, and leprosy [13]. With improved serum lupus serologies, a lupus band test is no longer considered a helpful test in determining whether a patient has SLE.
In order to properly diagnose cutaneous manifestations of LE, the physician must first correctly classify the subtype and exclude systemic involvement of the disease. As discussed earlier, diagnosis based solely on ACR criteria should be avoided, as the ACR criteria was designed to distinguish between the various autoimmune diseases. Rather, CLE diagnosis should be based on the findings of patient history, clinical exam, laboratory studies, serology, as well as histology and direct immunofluorescence (DIF) exam of skin biopsies if the histology is not diagnostic.
The more rare generalized form occurs above and below the neck, and has been referred to as a ‘maculopapular rash of lupus’ or ‘photosensitive lupus dermatitis.’ This presents as an often pruritic, widespread eruption of symmetric macules and papules that is photosensitive and may resemble a drug rash. Patients may have associated mucosal ulcerations/apthae, as well as diffuse hair thinning [14]. Generalized ACLE may resemble dermatomyositis as both diseases involve the dorsum of the hands, however, dermatomyositis affects the distal interphalangeal, proximal interphalangeal, and metacarpophalangeal joints, while they are spared in ACLE [13]. Cuticular overgrowth, as well as erythema or dilated vessels and drop-out of vessels in the periungual area are frequently seen. Lesions resembling erythema multiforme in ACLE or SCLE patients have been termed Rowell’s syndrome [15]. Rarely, a severe acute form can resemble toxic epidermal necrolysis. Other differentials include drug-induced photosensitivity, pemphigus erythematosus, atopic dermatitis, contact dermatitis, and photocontact dermatitis.
Upon completion of this course, you should be able to: 1 Identify those individuals at risk for developing systemic lupus erythematosus (lupus). 2 Compare and contrast the four types of lupus. 3 Evaluate the possible causes of lupus. 4 Identify common signs and symptoms of lupus. 5 Select the laboratory tests and diagnostic criteria necessary to appropriately diagnose lupus. 6 Analyze the various treatment options for lupus. 7 Discuss the impact of lupus as a chronic illness.
Compare and contrast the four types of lupus.
Systemic lupus erythematosus (lupus or SLE) is an autoimmune disease that has impacted the lives of more than 1.5 million individuals in the United States. Lupus is one of several chronic conditions that may result in long-term disability. The majority of SLE sufferers are young women. It is imperative that healthcare providers, including nurses, have a general knowledge base regarding this chronic disease. In addition, this knowledge base will provide healthcare providers with the tools necessary to provide appropriate care, guidance, and support for patients who are living with this chronic disease process.
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