Systemic lupus erythematosus (SLE) is a rare, multisystem autoimmune disease that can affect many parts of the body. It is most common in women ages 20-30. Symptoms include arthritis, skin rashes, fatigue, and kidney problems. Investigations include blood tests to check for autoantibodies. Treatment involves medications to reduce inflammation and suppress the immune system such as hydroxychloroquine, steroids, and immunosuppressants. Managing SLE requires a tailored treatment plan to control symptoms and prevent organ damage.

1. SYSTEMIC LUPUS
ERYTHEMATOUS
PRESENTED BY
ASHARA T
BOT 2018

2. CONTENT
 Introduction
 Pathophysiology
 Clinical features
 Investigations
 Medical management

3. INTRODUCTION
 SLE is most common multisystem connective tissue system.
 It’s a rare disease with prevalence that range from about 0.03 %.
 More commonly affected in female (90%), Age onset 20-30 years .
 SLE is associated with considerable to age and gender match control,because of
increased risk of premature CVD.

4. AETIOLOGY
– The cause is unknown, several predisposing factors are :-
– HERIDITARY :- higher concordance rate in monozygotic twins compared
with dizygotic twins.
– 3% chances of developing the disease in first degree relatives.
– 20% auto antibodies.
– GENETICS :- 20 genes linked to the development of SLE .
– These includes HLA, T and B lymphocytes.
– Homozygous deficiencies of the complement genes – c1q,c2 or c4

5. – SEX HORMONE STATUS :- premenopausal women are most
frequently affected.
– DRUGS :- hydralazine, isonaizid include form of SLE .
– UV RAYS

6. PATHOPHYSIOLOGY

7. CLINICAL FEATURES
Symptoms such as :-
• Fever
• weight loss
• Mild lymphadenopathy
• Fatigue
• Low grade joint pain

8. 1. ATHRITIS
2. KIDNEY
3. CARDIO VASCULAR
4. LUNGS
5. NEUROLOGICAL
6. HAEMATOLOGICAL
7. GASTROINTESTINAL
8. PAEDIATRIC DISEASE
9. Muscles and joints
CLINICAL SYMPTOMS

9. 1. ARTHRITIS
– Most common symptom – 90% people.
– Associated with morning sickness.
– Tenosynovitis, apparently synovitis with joint swelling.
– Joint deformities – jaccoud’s arthropathy.
– Tendon damage.

10. RAYNAUD’S PHENOMENON
– Common and antedate other symptoms by months or
years.
– It can be along with arthralgia or arthritis.
– Secondary Raynaud’s phenomenon associated with SLE
and other AICTDS .

11. – SEC. Raynaud’s phenomenon age onset of over 25
years, absence of family history and occurrence in
males.
– Capillary nail fall out :-diagnosis of SLE or severe
primary Raynaud’s phenomenon.
– If Raynauds phenomenon is severe digit ulceration can
occur.

12. 2.SKIN
– The skin is commonly involved.
– The main types involved are :-
TYPES
CLASSICAL /
MALAR
DISCOID
UTRICARIAL
ERUPTIONS
LIVEDO
RETICULARIS

13. UTRICARIAL ERUPTIONS LIVEDO RECTICULARIS

14. Classical facial rash
* Erythematous,
raised,painful,itchy.
* occurs over the cheeks with
sparing of the nasolabial fold.
* Rosacea is a mimic of this rash.
Discoid rash
Hyperkeratosis and follicular
plugging.
Scarring alopecia if it occur on the
scalp.
Livedo reticularis
Feature of antiphospholipid
syndrome.
Vasculitis if severe.

15. 3. KIDNEY
– Renal involvement is one of the main determinants of the prognosis .
– Regular monitoring of blood pressure and urine analysis is important.
 The typical renal lesions are. :-
proliferative glomerulonephritis
characterized by heavy heamaturia ,proteinuria ,casts on urine microscopy

16. 4. CARDIOVASCULAR
– The most common manifestation is pericarditis, myocarditis ,libman sacks
endocarditis .
– Endocarditis is due to accumulation on the heart valves of sterile fibrin
containing vegetations and also hypercoagulability with antiphospholipid
antibodies.
– The risk of atherosclerosis, stroke , myocardial infraction is increased.

18. 5. LUNGS
– Common and mostly frequently manifest as pleuritic pain( serositis) or
pleuritic effusion.
– Other features are pneumonitis, atelectasis , reduce pulmonary volume,
pulmonary fibrosis
– breathlessness.
– The risk of thromboembolism is increased.

19. 7. NEUROLOGICAL
– Fatigue
– Head ache
– Poor concentation
– Absence of laboratory evidence of active disease.
– More specific features include
– hallucination
– chorea
organic psychosis
Transverse myelitis
Lymphocytic menegitis

20. 8. HAEMATOLOGICAL
 Neutropenia
 Lymphopenia
 Thrombocytopenia
 Haemolytic anemia
 Due to antibody mediated destruction of peripheral blood cells.

21. 9. GASTRO INTESTINAL
– Mouth ulcers occur
– Peritoneal serositis can cause acute pain
– Mesenteric vasculitis present with abdominal pain , bowel
infection.
– Hepatitis rare feature.

22. PAEDIATRIC DISEASE
– Renal disease and cutaneous manifestation more common
– Juvenile onset of SLE .
– Higher incidence of renal, cutaneous vasculitis , Raynaud’s
phenomenon.

23. DIAGNOSTIC CRITERIA

24. INVESTIGATIONS
– BLOOD
– Blood count may show leucopenia .
– Auto immune hemolytic anemia .
– Increased ESR.
– Urea and creatine rise only when renal involvement is there.
– AUTO ANTIBODIES
– many different ANA Present.
– Anti- dsDNA, anti RO, anti SM common.
– Anti phospholipids antibodies are present.

25. – Serum compliment C3,C4 Are often reduced.
– Characteristics histological and immune fluorescent
abnormalities are seen in biopsies ( deposition of IgG).

26. MANAGEMENT
– MILD-MODERATE DISEASE
– Skin and joints can be maintained by analgesics, NSAIDS and
hydroxychloroquine.
– Glucocorticoids are also necessary ( prednisole )
– Combine with immune suppressants as methotrexate, azathioprine .

27. – SEVERE AND LIFE THREATENING DISEASE
– High dose of glucocorticoids and immune suppressants .
– Methyloprednisole + cyclophosphamide repeated 2-3 weeks .
– Myocophenole (MMF) succesfull high dodse glucocorticoids for
renal invovment.

28. MAINTANANCE THERAPY
– Typical maintenance through oral prednisole ( 40-10 mg)
– MMF
– The long term- low dose glucocorticoids because it cause cvs risk, renal, bp,
hyperlipidaemia etc….
– SLE Patient with risk of osteoporosis and hypovitaminosis D , should screen with
biochemistry and DXA scanning.

29. SYSTEMIC
SCLEROSIS

30. INTRODUCTION
– Systemic sclerosis is auto immune disorder of connective tissue.
– Which results in the fibrosis affecting the skin, internal organs and vasculature.
– The age onset is 4th and 5th decades
– overall prevalence is 10-20 per 100,000.
– female: male – 4:1

31. CLINICAL FEATURES
– Typically characterized by Raynaud’s phenomenon, digital
ischemia,sclerodactyly.
– Cardiac, lung, gut and renal disease.
TYPES
DIFFUSE
CUTANEOUS sscl
LIMITED
CUTANEOUS sscl

32. – Lcsscl- calcinosis and telangiectasia.
– 70% of cases
– Dcsscl- poor prognosis . Survival for 5 year
– 30% of cases
– include diffuse skin disease, proteinuria, high ESR,
– pulmonary hypotension, low transfer factor for co (TLCO)

33. PATHOPHYSIOLOGY

34. CLINICAL FEATURES
SKIN
RAYNAUDS
PHENOMEN
ON
MUSCULOSKE
LETAL
FEATURES
GIT PULMONARY RENAL

35. SIGNIFICANT CLINICAL FEATURES

36. 1. SKIN
– Initially there is no pitting edema of finger and flexor tendon sheaths.
– Skin becomes shiny and taut , distal skin creases disappear.
– Capillary loss.
– Face and neck are involved.
– Thinning of lips and radial furrowing.
– Lcsscl- skin involvement restricted to knees or elbow ( apart from face)
– Dcsscl- diffuse disease , involvement of elbow, knees, trunk.

37. 2.MUSCULOSKELETAL
FEATURES
– Arthralgia and tenosynovitis is common.
– Restricted hand function.
– Muscle weakness and wasting can be results from myositis .

38. 3. GIT
– EROSIVE OESOPHAGITIS :-smooth muscle atrophy and fibrosis on the lower 2/3rd
– Dysphagia and odynophagia , satiety.
– Recurrent upper GIT bleeding- watermelon stomach .
– Small intestine leads to malabsorption- bacterial over growth
– Vomiting, nausea, abdominal pain , autonomic neuropathy.

39. 4. RENAL INVOLVEMENT
– One of the main cause of death is hypertensive renal crisis, renal
failure.
– Characterstic feature of Dcsscl.

40. INVESTIGATIONS
– As sscl affect multiple organ , routine haematology ,renal, bone
fuction test and urine analysis are essential.
– ANA is positive.
– Chest x ray ,trans thoracic echocardiography and ling function .
– Blood pressure .

41. MANAGEMENT
– No treatment are available that halt or reverse the fibrotic changes .
– Raynaud’s phenomenon-
– avoiding of cold exposure , use of thermal insulating gloves/stocks.
– If symptoms are persistant ca channel blockers , losartan are efficacy.
– Intra venous prostacyclin are used for severe disease & critical ischemia .

42. – HYPERTENSION :- aggressive treatment with ACE inhibitors.
– JOINT INVOLVEMENT :- treated with analgesics /NSAIDS. If synovitis present
and both RA ( OVERLAP CONDITION) low dose methotrexate can be used.
– PROGRESSIVE PULMINORY HYPERTENSION :- bosentan, severe- heart lung
transplantation.
– INTERSITIAL LUNG DISEASE :- glucocorticoids and cyclophosphamide.

43. POLY ATHRITIS

44. INTRODUCTION
– This term is used to describe pain and swelling affecting five or more joints or joint group
– Polyarthritis can present as acute episodes or it may become chronic, lasting for more
than six weeks.
– Polyarthritis can follow many viral infections. It may evolve into a specific type of
autoimmune disease, such as rheumatoid arthritis, lupus, or Sjogren’s syndrome.
– Women are more affected than female .

45. TRIGGERS ARTHRITIS
RHEUMATOID ARTHRITS
VIRAL ARTHRITIS
OSTEO ARTHRITIS
PSORIATIC ARTHRITIS
ENTEROPATHIC
ARTHRITIS
JUVENILE IDIOPATHIC
ARTHRITIS

46. RHEUMATOID ARTHRITIS VIRAL ARTHRITIS
Symmetrical pain
Small and large joints
Upper and lower limb.
Symmetrical
Small joints
Rash and prodromal illness
Self limiting.

47. OSTEO ARTHRITIS PSOARTIC ATHRITIS
Symmetrical
Pip, dip, cmc joint in hand
Knees ,hip, back and neck.
Heberden,s and bouchard’s nodes.
Asymmetrical
All joints
Associated with nail pitting
dactylitis

49. JUVENILE IDIOPATHIC ARTHRITIS ENTEROPATHIC ARTHRITIS
Various pattern
Poly articular
Oligo articular
Systemic symptoms
Chronic inflammatory
Bowel disease
Inflammation of peripheral joints
Abdominal discomfort.

51. CLINICAL FEATURES
– The most important diagnosis to consider are PSA, RA and inflammatory small
joint.
– RA is characterized by symmetrical involvement of the small joints of the
hands ,feet, wrist, ankles, knees.
– Poncet’s disease – in region where tuberculosis is highly prevalent.
– Asymmetry , lower limb predominance .
– Enthesis and greater involvement joints are the characterstics of SPAs.
– PSAs involvement of proximal and distal interphalangeals.

54. INVESTIGATION
– Blood samples taken routine haematology, biochemistry;
– ESR , CRP , Viral serology;
– Immunological screening include ANA , RF, and ACPA .
– Ultrasound and MRI presence of synovitis

55. MANAGEMENT
– Treatment with non steroidal anti inflammatory drugs.
– Analgesics.
– Systemic glucocorticoids can be considered if symptoms are very severe or
having great functional impact.
– Early immunotherapy (DMARDs) is required in RA & some cases in PSA .
– Early accurate and specific diagnosis is very important

56. REFERENCES
– Harrison’s principles of internal medicine
18th edition.
- Davidson’s essential's of medicine
23rd edition .
- Kumar and Clark clinical medicine
7th edition
- Gowalla's medicine for students
Sharukha A GOLWALLA