Systemic Lupus Erythematosus (SLE) Diagnosis Symptoms and Treatment

Systemic Lupus Erythematosus (SLE) Diagnosis Symptoms and Treatment

SystemPneumothorax Symptoms Causes Diagnosis Treatment with emergency. Read more ... »ic Lupus Erythematosus (SLE)

Systemic Lupus Erythematosus (SLE) is an au­toimmune diseasePneumothorax Symptoms Causes Diagnosis Treatment with emergency. Read more ... » of unknown etiology in which there is tissue damage by pathogenic antibod­ies.

Multisystem autoimmune inflammatory disease characterized by fluctuating, chronicChronic Hepatitis Classification viral hepatitis Clinical features and Treatment. Read more ... » course; varies from mild to severe and may be life-threatening (CNS and renalDiabetic Nephropathy and Renal complications of DM (Diabetes Mellitus). Read more ... » forms)

chronic autoimmune inflammatory disease involving multiple organ systems and marked by periodic acutePneumothorax Symptoms Causes Diagnosis Treatment with emergency. Read more ... » episodes.

System(s) affected: Mucocutaneous; Musculoskeletal; Renal; Nervous; Pulmonary; CardiacAcute Myocardial Infarction (AMI) Causes PATHOPHYSIOLOGY and Etiology. Read more ... »; Hematologic; Vascular, GI

Its name is derived from the characteristic erythematous “butterfly” rash over the nose and cheeks, which resembles a wolf’s snout.

Synonym(s): SLE, lupus

SLE ClinicalPneumothorax Symptoms Causes Diagnosis Treatment with emergency. Read more ... » features Symptoms –

SLE History

SLE Physical Exam

SLE Pathogenesis

  • Tissue damage by autoantibodies and immune com­plexes depends on :
  • 1. T and B Iymphocyte hyperactivity
  • 2. Susceptible genes
  • Genetic marker is – Class HI allele C4AQO.

SLE Clinical manifestations Symptoms

  • · Fatigue
  • · Malaise
  • Fever
  • Anorexia
  • Weight loss Arthralgias Myalgias, myopathy Arthritis
  • - Severe pain and swelling in joints-hands,
  • wrists, knees affected.
  • Puffiness, tenosynontis, swan-neck deformities in 10%.
  • Sub-cutaneous nodules.
  • · Myopathy due to : - Hypokalemia
  • - GlucocorticoidsAdrenal Gland Steroids. Read more ... »
  • - Hydroxychloroquine.
  • · Ischemic necrosis of bone – hip, knee, shoulder pain due to glucocorticoids.
  • · Skin: Butterfly rash, photosensitive, fixed erythematous (red) rash, over cheeks, bridge of nose, ears and chin ·even.
  • Sometimes diffuse maculopapular rash, sun­burns. There is no scarring and loss of hair.

DLE (Diffuse lupus erythematosus)

  • · DLE in 20% of patients
  • · DLE is disfiguring
  • · There is central atrophy and scarring of lesions
  • · There is loss of hair
  • · Occurs over scalp, ears, face, arms, back, chest (exposed areas only).

Less frequently Urtcaria Bullae

Erythema multiforme Lichen planus Panniculitis.

SLE SCLE (Subacute cutaneous lupus erythematous) Vasculitic skin lesions seen in SLE, DLE or SCLE.

  • · Purpura
  • · Sic nodule
  • · Nail-fold infarcts
  • · Ulcers
  • · Vascular urticaria
  • · Gangrene.
  • Ulcers in nose and mouth in SLE.

 

SLE Renal manifestations

e OUS!i S em

  • There is involvement of : – Meninges
  • - Spinal cord
  • - Cranial and peripheral nerves.
  • Some of the presentations are
  • · Cognitive dysfunction
  • · HeadacheHeadache. Read more ... »
  • · Seizures
  • · Psychosis
  • · Confusion
  • · Demyelinating disorders
  • · Cerebrovascular disease
  • · Movement disorders
  • · Aseptic meningitis
  • · Myelopathy
  • · Mono-polyneuropathy (GB syndrome)
  • · Mood disturbance
  • · Optic neuritis
  • · Subarachnoid haemorrhage
  • · Pseudotumor cerebri
  • · Inappropriate secretion of vasopressin
  • · Depression, anxiety.

Lab diagnosis:

  • Abnormal EEG CSF (cerebro spinal fluid) :
  • Increased protein levels Increased monocyte Increased Ig.
  • Lumbar puncture / CSF not routine. MRI can diagnose focal lesion.
  • CT Scan for detection of mass or haemorrhage. Angiograms for vasculitis, and occlusions and emboli
  • Treatment
    • Immunosuppressives Recurrence in 1/3 of patients.

I cular s ste

  • Antibody against phospholipids
  • · Lupus anticoagulants (LA)
  • · Anticardiolipin (aCL).
  • Lesions of Libman Sacks endocarditis may embolize to brain.
  • Degenerative cerebral and coronary artery disease. Hyperlipidemia is side effect of long term steroid therapy
  • Treatment:
    • Anticoagulation, with or without immu­nosuppression.
  • Manifestations
    • · Anaemia
    • · Hemolysis : +ve Coomb’s test Treatment
    • - High dose Glucocorticoids, Splenectomy for refractory cases.
    • · Leukopenia (No treatment)
    • · Thrombocytopenia - Bleeding, Purpura Treatment
    • - Steroids in high dose.
    • - IV Gamma globulin gives short term response.
    • - Addition of cytotoxic drugs after 2 weeks.
    • - If no response: Cyclosporin, Danazole, Splenectomy.

SLE Diagnosis

  • Russell viper venom tincture for LA (LUpus antico­agulants).
  • ELISA tests

    • Lupus anticoagulants in
    • · Thrombocytopenia
    • · Recurrent arterial clotting
    • · Recurrent venous clotting
    • · Recurrent fetal loss
    • · Valvular heart disease
    • · Bleeding
  • Diagnostic Procedures

    • Renal biopsy to diagnose lupus nephritis
    • Skin biopsy with immunofluorescence on both involved and uninvolved non-sun-exposed skin (lupus band test) may help to differentiate SLE rash from other disorders.
    • Lumbar puncture in patients with fever and CNS or meningeal symptoms
    • EEG for seizures or global CNS dysfunction
    • Neuropsychiatric testing for cognitive impairment
    • EMG/NCS to diagnose peripheral neuropathy and myositis
    • Nerve and/or muscle biopsy
    • ECGAcute Myocardial Infarction (AMI) Causes PATHOPHYSIOLOGY and Etiology. Read more ... », cardiac enzymes, stress tests
  • Treatment: 
    • glucocorticoids for bleeding

SLE Pulmonary and cardiac manifestations

SLE Gastrointestinal system

Ocular manifestations Retinal vasculitis:

    • · Sicca syndrome
    • · Conjunctivitis
    • · Episcleritis
    • · Optic neuritis.
  • Lab diag osis of SLE Screening test
    • · ANAs (non specific) (Anti-nuclear anti bodies) – >95% patients +ve (non specific)
    • - (High serum ANA)
    • · dsDNA (Double stranded DNA)
    • · Decreased complement
    • · Urine analysis:
    • - Hematuria. casts and protein
    • · Serum creatinine increased.

Pregnancy and SLE

  • · H/o repeated abortions
  • · Give low dose heparin 5000 U sic BD
  • · Low dose aspirin may be given
  • · Low dose corticosteroids may be given
  • · Disease may flare up in 6th week postpartum
  • Neonatal lupus -
    • may have skin rash and congeni­tal permanent heart block.
    • Survival in SLE is <75% at 20 years.
Bad Signs
  • · Increased serum creatinine >1.4 mg/dl
  • · Hypertension
  • · Anaemia
  • · Nephrotic syndrome
  • · Hypoalbuminemia
  • · Hypocomplementemia
  • · Thrombocytopenia
  • · Thromboembolic events.

Pregnancy and SLE Treatment

  • No cure Glucocorticoids
  • NSAIDs (cox 2 inhibitors)
  • Antimalarials – 400 mg HCQ for few weeks (needs regular eyeOphthalmologic Complications of DM (Diabetes Mellitus). Read more ... » checkup)
  • Intralesional steroids
  • Oral dihydroepiandrosterone
  • High dose steroids 1-2 mg/kg per day (QID) a tapered
  • 3-5 days of methylprednisolone 1000 mg Look for steroid side effects
  • Treat infections
  • Calcium
  • Vitamin D
  • HRT – HormoneAnterior Pituitary Hormones and Disorders. Read more ... » replacement therapy with es­trogen
  • Calcitonin.
  • Cytotoxic agents
    • Azathioprin Methotrexate Clorambucil Cyclophospha mide
  • Plasmapharesis
  • Anticoagulation -
    • for Antiphospholipid syndrome .
  • Cyclosporin
  • Intravenous immunoglobulins Autologous stem cell transplantation

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