1. SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)

2. DEFINITION
Systemic lupus erythematosus is a multi-system autoimmune disease which is characterized by production of antinuclear antibodies.
SLE is a prototype of human immune complex disease.

3. INCIDENCE AND PREVALENCE
Incidence – 4 to 7 new cases per 100,000 per year
Prevalence – 1:2,000 population
Age – Peak onset 15 to 45 years old
Female:Male ratio – 4 to 10 :1
Black:White ratio – 3:1
Prevalence in all women ages 15 to 64 is 1/700 Prevalence in black women of same age is 1/245
Other populations commonly affected:
Hispanics
Chinese

4. Pathogenesis and pathophysiology

6. Familial/Genetics/HLA

10. Common Clinical Manifestations Of SLE
Manifestation Approximate Frequency
Arthralgia/Myalgia
Fatigue
Fever
Central Nervous System
Skin Changes
Anemia
Adenopathy
Gastrointestinal
Renal Changes
Pericarditis
Leukopenia
Pleural Effusion
Arthritis

11. 1982 CLASSIFICATION CRITERIA SLE
Serositis
Oral ulcers
Arthritis
Photosensitive rash
Blood dyscrasias
Renal disorder
ANA
Immunologic disorder
Neurologic disorder
Malar rash
Discoid rash
** SLE if 4 of 11 present serially
or simultaneously

12. SKIN INVOLVEMENT
ACUTE
SUBACUTE
CHRONIC

13. MALAR RASH
Fixed erythema
Flat or raised
Spares the nasolabial folds

14. MALAR RASH

18. Lupus band test

19. DLE Plugging atrophy scar telangiectasia erythematous raised rim
above trans-nipple line

20. DISCOID RASH Raised Patches Adherent Keratotic Scaling
Follicular Plugging
Older Lesions May Cause Scarring

22. Balloon degeneration of epidermal cells

23. LE-nonspecific Skin Lesions
Panniculitis
Urticarial lesions (5-10%)
Vasculitic lesions
Raynaud’s phenomenon (50%)

24. Lupus Paniculitis

28. OTHER SLE RASHES Palmar Erythema and Rash
Subacute Cutaneous Lupus Erythematosus (SCLE)
Bullous Lupus Dermatitis
Neonatal Lupus
Raynaud’s Phenomenon

30. Subacute Cutaneous Lupus Erythematosus (SCLE)

31. Bullous Lupus Dermatitis

32. Neonatal Lupus

33. ORAL ULCERS Can affect all mucosal surfaces
Painless (which distinguishes them from aphthous ulcers)

34. ORAL ULCER

35. ALOPECIA

36. SEROSITIS
Pleuritis,
Pericarditis or
Peritonitis.

37. ARTHRITIS Clinically indistinguishable from RA Nonerosive Symmetric
Inflammatory
> 2 peripheral joints

38. Arthritis (Non-erosive, usually symmetric)
Bone & Joints in SLE
Arthralgia
Arthritis (Non-erosive, usually symmetric)
Nodules (10%)
Tenosynovitis
Jaccoud’s joint
Osteonecrosis
Osteoporosis

40. Jaccoud’s Arthropathy

41. PHOTOSENSITIVITY
Skin Rash From UV irradiation

42. BLOOD DYSCRASIAS Hemolytic anemia with reticulocytosis or
Leukopenia- <4,000 WBC
on > 2 occasions or
Lymphopenia- <1,500 on 2 occasions or
Thrombocytopenia- <100,000

43. RENAL DISORDER Persistent proteinuria > 0.5 grams/day or
Cellular casts or > 10 RBC/HPF

44. LUPUS RENAL HISTOLOGY WHO designation:
I: Normal light microscopy, but immuo globulin and /or complement deposits
II: Mesangial Lupus Nephritis
III: Focal Proliferative Glomerulonephritis
IV: Diffuse Proliferative Glomerulonephritis
V: Membranous Nephritis

45. RENAL INVOLVEMENT 1.Msangial Glumerolonefritis(2)
2.Focal Proliferative Glumerolonephritis(3)
3.Diffuse Proliferative Glumerolonephritis(4)
4.Memberanous Nephritis(5)
5. Glumerolosclerosis(6)
7.Tubulointerstitial Nephritis
8.Infection
9.Drug-Induced Nephritis

46. Lupus Nephritis: Manifestations
Proteinuria: ~100%
Nephrotic syndrome: 50%
Granular casts: 30%
Red cell casts: 10%
Microscopic hematuria: 80%
Macroscopic hematuria: 1-2%
Reduced renal function: 40-80%
Hypertension: 15-50%

47. Class II: Pure Mesangial
Mild to moderate proteinuria and hematuria.No HTN
Little or no evidence of renal insufficiency.

48. Class III: Focal Proliferative GN
Less than 50% of glomeruli and less than 50% of each glomeruli are involved.
Subendothelial deposits
clinically:

49. Focal Proliferative Glomerulonephritis

50. Class IV: Diffuse Proliferative GN
The most severe form of lupus nephritis.
Along with class III, makes >50% of cases
subendothelial deposits
clinically:

51. Diffuse Proliferative Glomerulonephritis

52. Class V: Diffuse Membranous GN Subepithelial deposits5 - 10 %
of all •
lupus
nephritides.
Presents •
mainly with massive
proteinuria.
Subepithelial deposits

53. Membranous Glomerulonephritis

54. Subepithelial deposit in Membraneous GN

55. SLE & Respiratory System
Pulmonary Disease
Acute lupus pneumonitis
Alveolar hemorrhage
Chronic ILD
Lymphocytic pneumonia
Bronchiolitis obliterans
Respiratory Muscle Disease
Shrinking lung syndrome
Pleural disease
Pleuritis
Upper Airway disease
Epiglotitis
Subglotic stenosis
Laryngeal edema
Laryngeal ulceration
Cricoarythenoid arthritis
Necrotizing vasculitis
Vascular Disease
Pulmonary hypertension
Pulmonary embolism
Acute reversible hypoxemia

56. GI INVOLVEMENT Peritonitis Vasculitis Pancreatitis
IBD( collagenous colitis)

57. NEUROLOGIC DISORDER
Seizures
Psychosis

58. NEUROPSYCHIATRIC MANIFESTATIONS
9.Aseptic Meningitis
10.MS-like Disorder
11.Guillan-Barre Syn.
12.SAH
13.Ataxia
14.Dementia
15.Psychiatric Disorders
1.Headache
2.Seizure
3.Stroke
4.Chorea
5.Neuropathy
6.Vertigo
7.Tinnitus
8.Transverse Myelitis

59. CNS LUPUS

61. Antinuclear antibodies

62. ANTINUCLEAR ANTIBODY Significant titer is > 1:80
Found in 95% SLE patients but also-
Interstitial pulmonary fibrosis
MCTD
PSS
Polymyositis
Procainamide/Hydralazine/Dilantin RA
Sjogrens syndrome
Normal population

63. ANA - RIM PATTERN

64. ANA - HOMOGENOUS

65. ANA - SPECKLED

66. ANA - NUCLEOLAR

68. LE-cell
LE-cell is an in-vitro phenomenon where a PMN phagocytoses the antibody-coated nucleus of a cell. The technique is an insensitive way to detect anti-nuclear antibody in a serum specimen.

69. Hematoxyllin Body
Hematoxyllin body is the in-vivo counterpart of LE cell.
It is considered pathognomonic for SLE lesions.

70. IMMUNOLOGIC DISORDER anti-ds DNA antibody (anti-native DNA ab) or
anti-SM antibody or
antiphospholipid antibody based on either:
abnormal serum IgG or IgM anticardiolipin ab or lupus anticoagulant or
false positive RPR or VDRL for > 6 months and
confirmed by t. Pallidum immobilization or fluorescent treponemal antibody (FTA)
absorption test.

72. APS
IgG ,IgM APL
LA assay
VDRL
hypercoagulable state

74. LEIBMAN-SACHS ENDOCARDITIS

75. Prognosis Creatinin > 1.4 HTN NS(> 2.6 g/24h) anemia
low C and albomin
low socioeconomic state
major organ involvement
APS+
thrombocytopenia

77. Mortality Infection & renal involvement in 1st decade
Thromboembolic phenomena in the 2th.

78. Treatment NSAIDs antimalarials sunscreens(SPF >15) androgens (DHEA)
immuno-suppression
anti-thrombotics & anti-platelets
IvIG
BMT

79. THERAPY Serositis NSAID Arthritis NSAIDs Blood dyscrasias
Corticosteroids
Immunosuppressive
Arthritis NSAIDs
Hydroxychloroquine
Immunosuppressive Drugs
Blood dyscrasias

80. THERAPY cont. Renal Corticosteroids Neurologic Symptomatic
Immunosuppressive
Neurologic Symptomatic
Corticosteroids
Rash Topical Corticosteroids
Systemic Steroids
Hydroxychloroquine
Immunosuppressive Drugs