RHEUMATOLOGY (WWW.MEDICINENOTES.

IN) 2013

Systemic lupus erythematosus

IMPORTANT POINTS These notes are made from Standard Textbook of Medicine The current notes corresponds to Chapter 319 of Harrisons Internal medicine The Yellow shades refer to things you have to revise frequently (atleast once a week) The Blue shades refers to concepts The Pink shades refers to concepts that are not required for undergraduate level but useful for Postgraduate level.

Q. True or False- SLE is more common in females True SLE is a disease common in young females

Q. Which HLA classes have increased risk of Lupus ? HLA DR2,3 and 8 have high risk of SLE CLINICAL MANIFESTATIONS OF LUPUS Q. What is American College of Rheumatology criteria for Diagnosis of SLE ? ACR criteria is 4 of 11 must be fulfilled- Mneomic is MAD HAIR NORP 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. Malar rash Arthritis Discoid rash Hematological criteria- Leukopenia (<4000), lymphopenia (<1500), Thrombocytopenia (<1 lakh) Anti-Nuclear antibody Immunological criteria- Anti DS DNA, Anti SM, Antiphospholipid antibody Recurrent Serositis Neurological criteria- Seizures or Pyschosis without any other cause Oral ulcers Renal disorder Photosensitivity

Q. SLE is a disease of Exacerbation and remission, true or false ? True. SLE is disease characterised by exacerbation and remission However patient is rarely completely alright Q. Which are the systemic symptoms of SLE ? DR. OM LAKHANI, MD (WWW.MEDICINENOTES.IN) Page 1

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Systemic symptoms include Fever Arthralgia Myalgia Anemia Prostration Weight loss

Q. True or False: SLE produces Erosive arthritis ? False. SLE is mainly associated with non erosive arthritis. Typically involves single joints. Hands, wrist and Knee commonly involved.

Q. Which are the different skin lesions in SLE ? Skin lesions in SLE are Discoid Lupus

Subacutecutaneous lupus erythematosus (SCLE) Systemic Rash Others

Q. Which antibody is classical associated with SCLE ?

SCLE is associated with Anti-Ro antibody Q. Do all patient with DLE have SLE ?

No. 5% of patients with DLE have SLE while 20% of patients with SLE have DLE. Q. What is the classical description of rash in SLE ?

Rash in SLE is described as a Butterfly rash (LUPUS NEPHRITIS- SEE NEPHRITIC SYNDROME CHAPTER)

Q. What are the various neurological manifestations of Lupus

1. CNS Lupus- cognitive decline and decline memory and reasoning difficult 2. Headache severe headache suggest a lupus flare DR. OM LAKHANI, MD (WWW.MEDICINENOTES.IN) Page 2

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3. 4. 5. 6. 7. Myelopathy Siezures Pyschosis Steroid induced pyschosis- Resolves after stopping steroids Increase risk of Stroke and TIA

Q. What is the reasons for increase risk of Vascular events associated with SLE ? SLE patient are often having associated APLA syndrome (Antiphospholipid antibody syndrome) which is a prethrombotic state and associated with acute vascular events. SLE patients also have accelerated atherosclerosis due to chronic inflammatory status. They sometimes also have vasculitis associated with it Q. What are the cardiac manifestations of SLE ? Cardiac manifestations of SLE are Libman Sacks endocarditis Lesion on lower surface of valve. Mitral and aortic valvues involved Pericarditis- most common manifestation- low risk of Tamponade Myocarditis Increase Risk of Myocardial infarction- 7-10 times higher risk.

Q. Which are the pulmonary manifestations of SLE ?

Pleuritis +/- Pleural effusion commonest pulmonary manifestation of SLE Shrinking lung syndrome Intralverolar hemorraghe Interstitial fibrosis

Q. What are the hematological manifestations of SLE ? Anemia- due to chronic disease or because of hemolysis Lymphocytopenia (no granulocytopenia)- low risk of infection Thrombocytopenia- treatment required if o Abnormal bleeding time o Platelet count <40,000 Q. True or False. Patients with SLE have high risk of infertility ? False. SLE is not associated infertility. However if APLA syndrome is associated , it is associated with increase risk of recurrent fetal loss. Low dose of steroids are recommended in patients with SLE during pregnancy. Patients with APLA syndrome are given Asprin plus heparin (or LMWH) during pregnancy to prevent fetal loss. DR. OM LAKHANI, MD (WWW.MEDICINENOTES.IN) Page 3

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LAB INVESTIGATIONS Q. A patient has repeatedly negative ANA, would you consider him having SLE ? No ANA is a good screening test for SLE. Negative ANA rules out SLE Q. Which antibodies are specific for SLE ? Anti dsDNA and anti SM antibody are two most common antibodies associated with SLE.

Q. Which autoantibody is associated with Lupus nephritis ? Anti dsDNA is specific for lupus nephritis Anti Ro (SS-A antibody) and Anti La (SS-B) rules out Lupus nephritis. Q. Which is the marker of Overlap syndrome ? Anti RNP antibody suggests an Overlap syndrome

Q. Which is the antibody for Neonatal Lupus ? Anti Ro antibody is associated with Neonatal lupus and congenital heart blocks. Q. Which is the antibody associated with drug induced Lupus ? AntiHistone antibody associated with drug induced lupus Q. Which are the three tests done for Antiphosphopholipid antibody (APLA syndrome) ? The three tests for ALPA syndrome are 1. Lupus anticoagulant 2. Anti Cardiolipin antibody 3. Anti beta2 glycoprotein1 antibody Q. Which are antibodies associated with Cerebral Lupus ? Antibodies associated with Cerebral lupus are : Antiribosomal P antibody Antineuronal antibody Q. What is the classical pathological finding in Lupus ? Classical pathological finding is deposition of Ig at Dermal Epidermal junction

DR. OM LAKHANI, MD (WWW.MEDICINENOTES.IN)

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Q. What lab patterns suggest a possible flare of disease in SLE ? Reducing C3 with positive Anti-dsDNA suggest a possible flare of SLE

TREATMENT OF SLE

SLE

Non Lifethreatning

Life Threatning

NSAIDS

HCQ

Induction of Remission - IV steroid + Cyclophosphamide

Maintanence of Remission- Oral Mycofenolate/ Steroids/ Azathioprine

Q. What is the role of Hydroxycholorquine in SLE ? HCQ reduces fatigue, dermatitis and arthritis in SLE It can cause Retinal toxicity so annual retinal examination is a must Dehydroepiandosterone in recent trials have shown some promise Some physician use low dose steroids in difficult to treat cases.

Q. Which is he newer Biological agent licenced for use in SLE ? Anti-BLyS (belimumab, directed against the ligand of the BLyS/BAFF receptor on B cells) is recent biological agent approved by US-FDA in SLE treatment

Q. What should be advised to patients with Skin lesion of SLE ?

DR. OM LAKHANI, MD (WWW.MEDICINENOTES.IN)

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Skin lesion of SLE are photosensitive hence patients are advised to apply sunscreen with SPF of atleast 15.

DRUG INDUCED LUPUS

Q. What are the difference between Drug induced lupus vs spontaneous SLE in terms of clinical presentation ? DRUG INDUCED LUPUS Not necessarily more in females Not involved Anti-Histone SPONTANEOUS LUPUS F>M May be involved Anti-ds DNA

Sex Kidney and CNS Antibody

Q. Name a few drugs implicated in Drug induced lupus ?

Drug induced lupus can remembered by mneomic SHIP MATS 1. 2. 3. 4. 5. 6. 7. 8. Statins Hydralazine Isoniazid Procainamide Minocycline Alpha methyldopa Thiazide diuretic Sulphasalazine

Q. What is the treatment of Drug induced lupus ? Drug induced lupus generally is self resolving with stopping of medications causing the problem

DR. OM LAKHANI, MD (WWW.MEDICINENOTES.IN)

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