SLE and Systemic Sclerosis

1. SystemicLupusErythematosus

2. Definition

3. Epidemiology

4. Pathogenesis

5. Pathogenesis

7. Pathogenesis

8. Clinical features

9. Clinical Presentation

10. Arthritis

11. Raynaud’s Phenomenon

12. Skin manifestations

13. Skin manifestations

14. Renal
Mesangial proliferative lupus nephritis
Advanced sclerosis lupus nephritisMembranous lupus nephritis

15. Cardiac

16. Cardiac

17. Respiratory

18. Neuropsychiatric
• Central Nervous System • Peripheral Nervous System
Neuropsychiatric syndromes
in SLE as defined by American
College of Rheumatology

19. Hematological

20. Gastrointestinal
Ocular
• Sicca syndrome (Sjogren’s)
• Conjunctivits
• Retinal vasculitis and optic neuritis – serious
complications

21. Diagnostic criteria

24. Useful Investigations

26. Useful Investigations
•Renal biopsy indicated in:

27. International Society of Nephrology
2003 classification of lupus nephritis
Class I Minimal mesangial Normal light microscopy
findings, abnormal electron
microscopy findings
Class II Mesangial
proliferative
Hypercellular on light
microscopy
Class III Focal proliferative <50% of glomeruli involved
Class IV Diffuse proliferative >50% of glomeruli involved
Class V Membranous Predominantly nephrotic
disease
Class VI Advanced sclerosing Chronic lesions and
sclerosis

28. Management
Mild to moderate disease
• Analgesics
• NSAIDS
• Hydroxychloroquine (200-400
mg/day)
• Corticosteroids
• Immunosuppressants – MTX,
Azathioprine, MMF
• Belimumab – shown to be effective

29. Life threatening disease
• High dose corticosteroids and
immunosuppressants
• Pulse methylprednisolone
(10mg/kg IV)
• Cyclophosphamide (15mg/kg IV)
• 2-3 weekly intervals – 6 cycles

30. Maintenance therapy

31. Medications in lupus nephritis
•Corticosteroids
•Immunosuppressive agents
(cyclophosphamide, azathioprine, or
mycophenolate mofetil)

32. •Hypertension:
•Hyperlipidemia

33. • Restrict protein intake
• Avoid nephrotoxic drugs
• Avoid pregnancy.
• Prevent Infection.
• Avoid sunlight exposure,
• Osteoporosis

34. Class specificTreatment
•Class I:
•Class II:

35. Class specificTreatment
•Classes III and IV:

36. Immunosuppressive drugs indicated in those :

37. Class specificTreatment
• ClassV:
• Immunosuppressive drugs are generally not used
effective in reducing proteinuria
• Rituximab:
• Infliximab:
• Abetimus:

38. Class specificTreatment
• End-stage renal disease

39. Prognosis
• In 1950s:
lupus nephritis
• Recently:
• In 1963:
• 1980-1990:
• 2004: J. Rheumatology

40. Prognosis
• Poor prognostic indicators

41. Prognosis
• Excellent prognosis:
• Good prognosis:
• Fair prognosis

43. Definition
1. Systemic sclerosis (scleroderma)
abnormalities of blood vessels
fibrosis

44. Epidemiology
1. Prevalence
2. Susceptibility: host factor
age 4th and 5th decade, declines
after menopause
female

45. Classification
1. Systemic sclerosis
2. Localized scleroderma

46. Classification of systemic sclerosis
1. Diffuse cutaneous systemic sclerosis
proximal extremity trunk and face
rapid progression
rapid onset
visceral involvement
poor prognosis

47. Classification of systemic sclerosis
2. Limited cutaneous systemic sclerosis
1) symmetric restricted fibrosis
- affecting the distal extremities and face/neck
2) prolonged delay in appearance of distinctive internal
manifestation
3) prominence of calcinosis and telangiectasia
4) good prognosis
* CREST syndrome
- calcinosis, Raynaud's phenomenon, esophageal
dysmotility, sclerodactyly, telangiectasia

49. Classification of systemic sclerosis
Overlap syndromes

50. Etiology
Environmental factors

51. Pathogenesis

52. Pathogenesis
Susceptible host
Exogenous events
Immune system
activation
Endothelial cell
activation/damage
Fibroblast activation
End stage pathology
Obliterative vasculopathy
Fibrosis

53. Pathogenesis
1.Vasculopathy of small artery and capillary

54. 2. Fibrosis

55. Clinical features
1. Vascular abnormalities
1) Raynaud's phenomenon
2) digital ischemic injury

59. Clinical features
2. Skin involvement
• Initially, non pitting edema of fingers, tendon sheaths
• Skin – shiny and taut, distal skin creases disappear +
erythema
• Face and neck involved  thinning of lips, radial
furrowing
• Decrease in range of motion, loss of facial expression,
inability to open mouth fully, contractures

63. Tight, thin lips with vertical perioral furrows

64. Thick skin of forearms
(proximal scleroderma)

65. Clinical features
3. Musculoskeletal system
• Arthralgia , morning stiffness and flexor
tenosynovitis (common)
• Erosive arthropathy - uncommon
• Muscle weakness and atrophy (primary /secondary)

71. GI involvement

72. Pulmonary Involvement

73. Pulmonary involvement

75. Cardiac
(10%)
1) Pericarditis
2) Heart failure
3) Arrhythmia
4) Myocardial fibrosis
Cardiac involvement – poor prognostic factor

76. Renal
• Chronic renal ischemia – shrunken glomeruli
• Acute scleroderma renal crisis  thrombotic microangiopathy –
reduplication of elastic lamina , intimal proliferation 
• Narrowing of lumen in small renal arteries  Thrombosis and
hemolysis
• C/F – accelerated HTN and progressive oliguric renal insufficiency
• Urine analysis – mild proteinuria, casts, microscopic hematuria,
fragmented RBC’s
• Oliguria or creatinine >3.0mg/dl – poor outcome

77. Other manifestations
Exocrine glands

78. Laboratory findings
nti-Scl-70 (DNA topoisomerase I) antibody
Anticentromere antibody

79. Diagnosis
one major 2 or more minor

80. Treatment
Wide spectrum of clinical manifestations and severity
- spontaneous improvement occurs frequently
• Disease modifying interventions
- penicillamine
- methotrexate – modest skin improvement
- mycophenolate mofetil
- immunosuppressive agent: cyclosporin, IFN-
- recombinant human relaxin
• Symptomatic (organ-specific) treatment

81. Treatment
Raynaud’s phenomenon and ischemia

82. Treatment
Gastrointestinal

83. Treatment
Pulmonary
1) Interstitial fibrosis
- corticosteroid
- cyclophosphamide, azathioprine
- pirfenidone and nintedanib
2) pulmonary artery hypertension
- Bosentan ( endothelin 1 receptor antagonist)
- PDE 5 inhibitor ( sildenafil )
- prostacyclin analogues – epoprostenol, treprostinil
- transplantation  if medical therapy fails

84. Treatment
Renal

85. Skin care

86. Musculoskeletal

87. Prognosis

88. Poor prognosis

89. MCTD – Mixed connective tissue
disease