Systemic Lupus Erythematosus

INTRODUCTION

Systemic Lupus Erythematosus is an autoimmune connective tissue disorder. This is the commonest connective tissue disorder which affects multi-systems. Majority (90%) of patients are women in child bearing ages But this can affect both sexes in all ages.

PATHOPHYSIOLOGY

In this condition Various antigentc stimulation causes wide spectrum of antibody production following B and T cell activation. The exact triggering factors for autoantibody production is unknown. Following this abnormal immune response. Antibodies or antigen-antibody complexes deposition in various organs causes influx of neutrophils and lymphocytes inducing inflammatory reaction. Ongoing same reactions finally leads to permanent organ damage.

CAUSES

History of hormone replacement therapy
Parimenopausal women in their child bearing ages are at risk of developing Systemic Lupus Erythematosus. Males with Klinefelter’s syndrome also at risk of developing this condition campare to normal males. Hormone replacement therapy is known to induce flareups.

Drug history
Some drugs like hydralazine, isoniazid, procainamide, penicillamine can induce this condition.

History of exposure to sun light
Ultraviolet light is a known triggering factor.

History of recent Epstein–Barr virus infection
Exposure to Epstein–Barr virus also a known predisposing factor.

Family history of diagnosed SLE or feature suggestive of SLE
Systemic Lupus Erythematosus goes as a hereditory disease. Deficiencies of the complement genes C1q, C2 or C4 are at risk of developing Systemic Lupus Erythematosus.

Past medical history
In a diagnosed patient complete past medical history will be useful in further management. About previous episodes, complications, tratments given, medications which patient is currently on.

CLINICAL FEATURES

Fever, weight loss and other constitutional symptoms like malaise, lethargy, fatiguability
These symptoms are common during exacerbation due to increased metabolic activity. But these features are not related to the severity of disease activity or complications.
Joint pain
Joint involvement is the main complain and this can be seen in more then 90% of patients. patient will complain of bilateral symmetrical small joint pain with mild morning stiffness. It can be a migratory arthralgia. Some times there will be swelling as well. Rarely there will be pain in major joints. these patients are at risk of developing aseptic necrosis of the hip/ knee as a rare complication of the disease or following the treatment with corticosteroids.
Development of severe pain and bluish discoloration over tip of fingers after exposure to cool wate
This called Raynaud’s phenomenon. These symptoms occur following vasculitis. These symptoms commonly associated with arthralgia or arthritis.
Muscle pain and weakness
around 50% of patients can develop myalgia but only few patients develop myositis.
Skin rash ( may be painful/ pruritic)
eruthematous rash on the cheeks aross the nasal bridge (butterfly rash). purpura and urticarial rash over the tip of the fingers and nail folds. These are caused by vasculitis (vasculitic rash). there will be other skin changes like Livedo reticularis and periungual erythema. Patient may also develop palmar and plantar rashes. Patients may develop a erythematous rash in face with well defined margins. there plques can ultimately lead to scarring and pigmentations (Discoid rash). In Subacute cutaneous lupus erythematosus, there is a migratory, non-scarring, papulosquamous/ annular rash.
Alopecia
Patients with disoid lupus, commonly developed scarred alopecia causing permanent skin loss (following discoid rash over the scalp).
Generalized body swelling, reduced urine output, haematuria/ frothy urine
Patients with renal involvement can develop these features of renal impairment. Thought histological changes of glomerulonephritis are occur in majority of patients with SLE only few will presennt with clinical symptoms.
Difficulty in breathing, pleuritic type chest pain
Patient will develop chest pain and difficulty in breathing following Pneumonitis, atelectasis. Patients also commonly develop bilateral exudative pleural effusions following pleuritis. With these lung manifestations there will reduction of lung volumes (shrinking lung syndrome). Rarely lung fibrosis can occur with Systemic Lupus Erythematosus. 50% people will have lung involvement. Intrapulmonary haemorrhages (due to vasculitis) can cause life threatening conditions.
cardiac involvement can be seen in 25% of cases. Pericarditis with pericardial effusion, myocaditis causing arrhythmias, valve lesions with cardiomyopathy, arterial and venous thrombosis following vasculitis can be seen. These patients are at risk of developing strokes and ischemic heart diseases.
Features of hypoperfusion/ ischemia following vasculitis
Due to the cardiac involvement and with the possibility of arterial and venous thrombus formation with vasculitis, patients are at risk of developing organ hypoperfusion. Eg: Brain strokes can occur giving features of paralysis, paresthesia and cranial nerve palsy. In spinal cord, features of infarction like limb paralysis, paresthesia, bladder/ bowel dysfunction. In bones, ischemic pain at site of the joint/back pain and fractures following long bone infarction can be seen. Myocardial infarctions can occur following hypoperfusion of the myocardium( chest pain, difficulty in breathing, dizziness). In lungs there will be shortness of breath and pleuritic type chest pain. In mesentry, acute abdominal pain will be the presentation. In digits, painful fingers and toes with small bone infarction. In kidneys infarction of medulla with papillary necrosis may lead to fail in concentrating urine causing high urine out put, dehydration and nocturnal enuresis. Chronic liver failure with micro infarction causing loss of appetite, yellowish discoloration of eyes. Splenic infarction leads to recurrent infections like upper/ lower respiratory tract infections and diarrheal illnessess.
Seizures, alteration in mental status, headache, poor concentration
60% of patient will develop cerebral lupus. theses patients are at risk of developing epilepsy, migraines, poor concentration, cerebellar ataxia, aseptic meningitis, cranial nerve lesions, cerebrovascular disease and polyneuropathy. Also there can be visual hallucinations, chorea, organic psychosis
Eye pain, redness of the eyes and visual impairment
With vasculitis there will be retinal infarctions leading to hard exudates, and haemorrhages. Also patients can develop episcleritis, conjunctivitis, optic neuritis and Sjögren’s syndrome (dry eyes and mucous menmbranes). Though several eye complications can occur, blindness is very rare.
Yellowish discoloration of the eyes, oral ulcers, sudden onset abdominal pain
patient with Systemic Lupus Erythematosus can develop oral ulcers, mesenteric ischemia (causes abdominal pain), bowel perforation (causes acute abdomen with sudden onset severe abdominal pain, and features of septicaemia), liver failure( right hypochondrial pain, icterus, loss of weight, loss of appetitie) and pancreatitis ( causing diabetes mellitus and features of food indigestion). In an acute flareup patient can develop nausea, vomiting and diarrhoea.
Recurrent infections, easy fatiguabilty, increased bleeding tendency
patients can develop neutropenia, lymphopenia, thrombocytopenia and haemolytic anaemia.

INVESTIGATIONS

  • Full blood count
    patients can develop neutropenia, lymphopenia, thrombocytopenia and anaemia (Anaemia of chronic disease or autoimmune haemolytic anaemia).
  • ESR, CRP
    In a controlled disease ESR will be high but CRP will be normal. But if there is any active inflammatory condition is present, CRP will be high.
  • Autoantibodies like ANA, anti-dsDNA, anti-Ro, anti-Sm and anti-La
    There are several antibodies present in Systemic Lupus Erythematosus. ANA are positive in more than 95% of patients.
  • Serum complement C3 and C4 levels
    These are normal during the remission but levels are low during flareups.
  • Renal function tests like UFR, serum creatinine, blood ures
    These tests help in diagnosing the renal involvement.
  • Liver function tests like AST, ALT, serum billirubin, serum albumin level and Ultrasound scan of the abdomen
    Liver function tests helpful in diagnosing the liver involvement and Ultrasound scan of the abdomen will help to assess the presence of ascitis.
  • ECG, Echocardiogram
    These will be helpful in diagnosing the cardiovascular complications like pericardial effusions, arrhythmias, and valvular abnormalities.
  • Chest X ray
    As Systemic Lupus Erythematosus can cause pulmonary involvement, Chest X ray is useful.
  • CT/ MRI scan of brain
    These tests will be useful in assessing cerebral complications. Brain arophy, brain infarction following vasculitis can be identified
  • Pleural aspirates for cytology, culture and ABST
    In the presence of pleural effusion these tests useful in identifyint the exudate anfd excluding pulmonary infections.
  • Histological and immunofluorescent study of biopsies from the kidney and the skin
    In these studies deposition of IgG and complement will be identified. This will be useful in classifying the lupus nephritis as well.
  • CSF studies like CSF full report, culture and ABST
    These will be helpful in excluding the septic meningitis in patients with clinical symptoms and signd suggestive of meningit

MANAGEMENT

  • Health education
    Patient should be educated regarding the disease, symptoms associated with, possible complications, investigations needed, available treatment options, predisposing factors for exacerbations and prognosis. As this is a life long genetically transmitting disease that fact also need to be address.
  • Life style modification
    Due to the symptoms associated with the disease patient’s normal day to day life can be affected. eg: malaise, lethargy, fatiguability will limit the patient to bed. Joint pain and deformities will interfere with fine works. This should be discussed with the patient and physiotherapy, occupational therapy and help from social services can be afforded to improve the quality of life.
  • Avoid precipitating factors
    Patient should be provide informations regarding precipitating factors like excessive exposure to sun light, drugs causing exacerbations and hormone replacement therapy. These factors should be avoided (eg: use high factor sun blocks to minimize UV light exposure) as much as possible and patient should be educated to early identification of exacerbations.
  • Non Steroid Anti Inflammatory Drugs
    In the presence of arthralgia, arthritis, serositis, fever and other constitutional symptoms standard doses of Non Steroid Anti Inflammatory Drugs will be useful.
  • Topical corticosteroids
    Topical corticosteroids are helpful in managing the skin conditions in cutaneous lupus.
  • Antimalarial drugs
    Antimalarial drugs like chloroquine, hydroxychloroquine can be used in mild skin disease, fatigue and arthralgias where those symptoms cannot be controlled with NSAIDs.
  • Corticosteroids
    Various types of corticosteroids can be use to treat this condition. eg: Short course of oral corticosteroids in mild to moderate disease condition like presence of rash, serositis. Single intramuscular injections of long-acting corticosteroids or short courses of oral corticosteroids are useful in treating severe flares of arthritis, pleuritis or pericarditis. High dose of oral corticosteroids in lupus nephritis, cerebral lupus, severe haemolytic anaemia or thrombocytopenia.
  • Immunosuppressive drugs
    in addition to high dose of oral corticosteroids in lupus nephritis and cerebral lupus patients should be treated with immunosuppressive drugs like Cyclophosphamide, mycophenolate mofetil. Azathioprine like immunosuppressive drugs are used in maintaining remissions.
  • Newer drugs like rituximab
    These are used in refractory cases of Systemic Lupus Erythematosus. It reduces the auto antibody levels by suppressing levels of CD20 positive B lymphocytes.
  • Warfarin
    Patients with past history of previous thrombotic complications should be given life-long warfarin.
  • Management of pregnancy In Systemic Lupus Erythematosus
    As mentioned above multidisciplinary team management should be considered. Pre- pregnant counseling about maternal risks (eg; increased risk of flareup, risk of development of hypertension, pre-eclampsia, placental abruption) and foetal risk (eg: pre term deliverty, pre term rupture of membranes, intra uterine growth retardation, intra uterine foetal death and development of neonatal lpus syndrome). advice to conceive during remissions as there is less risk of flare up. Treat hypertension and closely monitor for the foetures of pre eclampsis and imminent eclampsia. Treat flare ups similer to non pregnant state. advice patients not to stop hydroxychloroquine if patient is already on, as it can precipitate flare up. Manage the pregnancy in a hospital with tertiary care facilities available. closely monitor maternal and foetal well being.

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