Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that particularly affects women of childbearing age. The exact cause is still unknown, but a genetic predisposition, as well as hormonal and immunological influences, are considered likely etiological factors. The presentation of the disease is variable and may from mild localized symptoms to life-threatening systemic disease. Typical findings include and fatigue, a (facial '), myalgia, and arthritis. Any organ may be affected; however, damage to the or the nervous system is especially associated with a poor prognosis. The diagnosis of SLE is based on clinical findings and is further supported by tests, particularly for.

• Systemic Lupus Erythematosus Ppt

Management consists of supportive measures, such as avoiding sun exposure, and medication adapted to disease severity. Is administered in the majority of cases to maintain disease remission. For acute flares, are given as induction therapy, with dose and treatment duration adapted to the severity of the flare.

In severe cases, additional (e.g., mycophenolate, ) may be given. (LN). Description: most crucial prognostic factor in SLE.: common ( ∼ 50% of SLE patients ). Pathology: classically immune complex-mediated. Classification: classified according to differing grades of severity (WHO classes –VI).

Ranging from asymptomatic to. Ranging from asymptomatic to. Diagnosis. tests:, cellular casts (red cells, granular or tubular). biopsy: determination of disease severity. Treatment: Depending on the severity, cytostatic drugs (mycophenolate, ), and general measures to protect the (e.g., blood pressure control) may be required.

Comorbid conditions. Accelerated and cardiovascular complications (e.g., ). Pancytopenia.

Systemic Lupus Erythematosus Ppt

Does this patient have systemic lupus erythematosus? Systemic lupus erythematosus (SLE) is a systemic disease of unknown etiology in which immune responses are directed against the body’s own tissues. SLE is a disease whose hallmarks are autoantibodies and complement activation. While some autoantibodies are clearly pathogenic (i.e., anti-platelet antibodies, anti-lymphocyte antibodies), others (i.e., anti-dsDNA antibodies) form immune complexes that overwhelm the reticulo-endothelial clearance. Immune complexes deposit in target organs (i.e., kidney), activating the complement cascade, and inducing an inflammatory response. We recognise patients with SLE by constellation of clinical and laboratory abnormalities. Patients present with varied manifestations, some people experience only mild rashes and arthritis, others suffer debilitating fever, fatigue, joint pain, or severe organ and/or life-threatening disease.

This pleomorphic picture suggests a heterogeneous, rather than a unified, disease process. Classification criterion for SLE were originally developed by the American College of Rheumatology (ACR) in 1982 and revised in 1997. The ACR criteria contain 11 domains, and a patient needed at least 4 at any point in their lifetime. Concern that not all patients with SLE meet ACR criteria triggered the Systemic Lupus Collaborating Clinics (SLICC) group to revise and validate a second SLE classification criteria in 2012.

The SLICC criteria contained 17 domains, and patients needed to fulfill a minimum of four criteria, with at least one clinical criteria AND one immunologic criteria. Presence of biopsy proven lupus nephritis can be used as the sole clinical criteria in the presence of ANA or anti-dsDNA antibodies. Patients who do not meet the classification criteria may nevertheless have SLE since these criteria are not meant for diagnosis. In the strictest sense, SLE classification criteria standardises how patients can be included in research protocols.

Clinical and laboratory manifestations of SLE 1. Acute cutaneous lupus This includes the characteristic malar rash (or butterfly rash ), which is characterized by a facial fixed erythema that can be flat or raised and spares the nasolabial folds. Other lupus skin manifestations include maculo-papular rash, which is usually photosensitive. Sub-acute cutaneous lupus typically manifest with either raised annular rash or psoriasisiform lesions. Chronic cutaneous lupus This includes discoid rash, a localized when over the head, neck, scalp or ears or generalised if it occurs below the neck.

Discoid rash presents with keratotic scaly patches, with follicular plugging. Older discoid lesions can cause hyper- or hypopigmented scarring. When scarring occurs in an area with hair, alopecia is irreversible, while alopecia in non-scarring lupus dermatitis is reversible. Discoid lupus (and other types of cutaneous lupus) can be an isolated skin disease and does not have to be a component of SLE. Oral or nasal ulcers are usually painless and must be observed by a physician.

SLE ulcers are typically seen on the hard palate and should be differentiated from the commonly occurring aphthous ulcers which are painful and are located on the buccal mucosa. Alopecia patchy hair loss can occur in SLE especially in the setting of discoid disease.

However, diffuse and non-scaring alopecia is also very common in patients with SLE. Arthritis in SLE is typically non-erosive, non-deforming, and frequently precedes other manifestations of SLE. It is associated with morning stiffness, can be evanescent or persistent, affects the knees and the small joints of hands, proximal interphalangeal (PIPs), and produces objective evidence of inflammation (tenderness, swelling, effusion). It may result in reducible deformities referred to as Jaccoud’s arthropathy.

Serositis may present as painful or painless pleural, pericardial or ascitic fluid collections. However, only pleurisy and pericarditis are in the classification criteria. Serositis indicates inflammation of the lining of lung, heart and abdominal structures. Clinical features of a pleural/pericardial rub, effusion, or EKG changes are needed satisfy classification criteria.

Cardiac manifestations in SLE are common and are present in 25% of patients, comprising of: primary lupus carditis, pericarditis, myocarditis, endocarditis, conduction defects and secondary heart disease (ischemic, hypertensive systemic or pulmonary and infective). Kidney disease is defined by persistent hematuria (not in classification criteria), proteinuria, or cellular casts. This is one of the most common organ-threatening manifestations of SLE, clinically apparent in as many as 50% of patients.

In order to better understand the severity and prognosis of SLE nephritis, the World Health Organization (WHO) and more recently the International Society of Nephrology (ISN) proposed six classes of renal involvement in SLE based off of histopathologic findings as described below. Class I (Minimal mesangial lupus nephritis): Mesangial accumulation of immune complexes only seen by immunofluorescence, but normal glomeruli by light microscopy. Class II (Mesangial lupus nephritis): Mesangial thickening and hypercellularity with immune complexes confined to mesangium.

Normal capillary loops and tubules, and absent inflammatory cells. Class III (Focal proliferative lupus nephritis): 50% of glomeruli involved, diffuse hypercellularity of glomerular tufts with inflammatory cells, necrosis, sclerosis, and occlusion of capillary lumina (“wire loops”), capsular crescent formation, associated tubular atrophy, and extensive immune complex deposition in mesangium and subendothelial side of capillary basement membranes. Class V (Membranous lupus nephritis): Diffuse thickening of capillary walls, mesangial thickening due to increased mesangial matrix and mesangial cells, absence of inflammatory infiltrate and hypercellularity of capillary tufts, immune complex deposition confined to intramembranous and subepithelial area of capillary basement membrane (carries a slightly better prognosis but nonetheless needs aggressive therapy in order to prevent kidney damage).

Class VI (Advanced sclerotic lupus nephritis): 90% global glomerulosclerosis without any evidence of active glomerular disease. Class III and IV form a continuum of disease and carry the worst prognosis.

If left untreated, they progress to severe kidney damage, end stage renal disease, and the need for dialysis or renal transplantation. Interstitial disease may occur as well, though less common and as such not given a classification of its own. Neuropsychiatric SLE can be manifest in many different ways. Seizures or psychosis (organic mental syndrome), though rare, are the most characteristic central nervous system (CNS) manifestations of the disease and as such have been included in the classification criteria.

Other neuropsychiatric (NP) manifestations in SLE might include less specific features (such as headache, mood disorders and cognitive dysfunction) to rare events (such as seizures, psychosis and myelopathy). The reported prevalence of NP disease in patients with SLE has varied from 37% to 95%. Once organic disease has been excluded, functional brain disease requires traditional psychiatric care. The ACR recognizes 19 NP syndromes as primary manifestations of the disease. This requires that they are not the result of complications of the disease (e.g., hypertension), therapy (e.g., infection), or concurrent non-SLE-related NP event.

Current evidence suggests that NPSLE is a consequence of vascular abnormalities, autoantibodies, and the local production of inflammatory mediators. Antiphospholipid (APL) and anti-ribosomal P antibodies have shown the strongest clinical correlation with NP disease, whereas anti-N-methy-D-aspartate (NMDA) receptor antibodies associated with cognitive dysfunction. NPSLE represents a major challenge for clinicians, in view of the uncertainties surrounding the pathogenesis, diagnosis, and treatment. It is clear that the issue of NP disease in patients with SLE requires further investigation and there are major efforts underway to better understand it. Hemolytic anemia has been described in 10% of patients with SLE, this is usually direct Coombs positive. Patients with SLE can have other reasons for anemia including anemia of chronic disease and drug induced anemia.

Leucopenia WBC count of 10 mg/day to control symptoms. Antimalarial adjustment options for moderate flares might include maximizing hydroxychloroquine, addition or substitution with quinacrine or a switch to chloroquine. While these medications can help reduce symptoms, improve disease manifestations, and sometimes induce remission, they can also have significant negative side effects.

Steroids, in particular, commonly cause insomnia, osteoporosis, muscle weakness, and much more. Belimumab (Benlysta), a monoclonal antibody directed against a soluble B lymphocyte survival factor, has recently been approved for patients in this category. Severe flares Severe flares refer to life or organ-threatening disease, such as significant kidney disease, brain disease, very low platelet or red blood cell count, vasculitis. For such severe manifestations of SLE, treatment generally starts with pulse solumedrol (1 gram/day IV for 3 days), followed by high dose prednisone 1-2 mg/kg per day. More potent immunosuppressants, such as IV cyclophosphamide (Cytoxan), mycophenolate mofetil (CellCept), azathioprine (Imuran) or recently developed biologic therapies like Benlysta and rituximab (RTX) (trade name Rituxan) may be added. Renal disease The treatment of lupus nephritis (LN) has evolved over the last three decades paralleling an evolution in outcomes. Good quality studies have established that mycophenolate mofetil (MMF) is as effective as clyclophosphamide in the treatment of lupus nephritis, perhaps even more so in African American and Hispanic patients.

The first 6 months of therapy are the induction phase. During induction, the goal of therapy is to achieve renal remission which can be attained with the immunosuppressant MMF (2-3 grams daily in 2 divided doses) or the cytotoxic IV cyclophosphamide (CYC) (0.5-1 gram/m 2 monthly or 500 mg every 2 weeks for a total of 6 doses). Most SLE experts agree that the first episode of LN with normal creatinine should be treated with MMF and high dose steroids (1 mg/kg). This is especially true in younger patients of child bearing age. If the first LN episode has abnormal renal function, the patient might receive IV CYC with pulse steroids and high dose oral steroids. If there is no response to MMF or worsening nephritis at 6 months, the patient may then be treated with the National Institute of Health (NIH) IV CYC protocol plus steroids.

If there is still no response, RTX might be considered as a last resort. Non-immunomodulatory treatments should also be considered in the treatment plan for LN patients; these include ACE inhibitors, angiotensin receptor blockers (ARBs), and statins. Following induction, a maintenance phase typically 18 months of therapy aimed at preventing renal flares and deterioration of renal function.

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During maintenance, the therapeutic option is immunosuppression with MMF or azathioprine. It is unclear whether MMF is more efficacious than AZA (data from ASPREVA, MAINTAIN and Contreras is somewhat discordant). However, in clinical practice most clinicians prefer maintenance with MMF, except when pregnancy is contemplated or the cost is prohibitive. Guidelines for the treatment of SLE Nephritis have recently been published. Skin Disease Skin disease including alopecia can also be treated with topical and intra-lesional steroids in addition to hydroxychloroquine (HCQ) and other systemic therapies. UV protection is essential in all patients regardless of photosensitivity. IVIG and mycophenolate mofetil may be efficacious for refractory subacute cutaneous disease.

Serositis Both pleurisy and pericarditis respond well to non-steroidal anti-inflammatory drugs or glucocorticoids. Large volume effusions need therapy with 1 mg/kg of prednisone or equivalent. Occasionally, in addition to steroids massive effusions may benefit from drainage. Patients with cardiac tamponade due to SLE pericardial effusion require pericardiocentesis and drainage, or a pericardial window.

Recurrent serositis is managed with immunosuppressants. Raynaud's phenomenon Severe Raynaud’s syndrome may require medical therapy with calcium channel blockers, angiotensin receptor antagonists, or, in refractory cases, phosphodiesterase inhibitors, endothelin receptor blockers and Prostaglandin E2 inhibitors. Sicca symptoms A large number of patients with SLE suffer from sicca symptoms due to secondary Sjogren's syndrome in SLE. Moisture replacement therapies such as artificial tears may ease the symptom of dry eyes. Some have punctal plugs inserted to help retain tears on the ocular surface for a longer time. Additionally, cyclosporine eye drops (Restasis) are available by prescription to help treat chronic dry eye by suppressing the inflammation that disrupts tear secretion. Prescription drugs are also available that help to stimulate salivary flow, such as cevimeline (Evoxac) and pilocarpine.

Infection prophylaxis The guidelines for pneumocystis pneumonia (PCP) prophylaxis in patients with SLE are not well defined. PCP prophylaxis should be considered on patients who need 1 month of high dose steroids, or are on stronger immunosuppressive agents like Cytoxan (i.e. Induction therapy for lupus nephritis). Patients with SLE may have low lymphocyte counts either due to disease itself or secondary to medications, PCP prophylaxis should be considered in patients with a total lymphocyte count.