Artifacts in Nuclear Medicine with Identifying and resolving artifacts.
Systemic Sclerosis
1. Systemic Sclerosis Samar Tharwat Radwan
Lecturer of Internal Medicine
(Rheumatology & Immunology)
Mansoura University
2. Definintion
Systemic sclerosis
• A multisystem connective tissue disease
• Affect the skin and internal organs
• Chronic inflammation +fibrosis+ vasculopathy
3. Incidence and Prevalence
• A rare disease
• Incidence:18-20 cases per million population per year
• Prevalence: 100 -300 cases per million population
• Age:35 -50
• More common among women (3 to 7 : 1 female-to-male ratio)
4. General Principles
• Early, signs of classic inflammatory events
• Later, indolent subclinical fibrotic process organ damage
• Skin is the site of the most dramatic physical findings
• Multisystem disease
10. Skin Manifestations in SSc
Raynaud’s
phenomenon
Telangiectasias Ulcer Hypopigmentation
with salt and-pepper
Spotty
hypopigmented areas
Thickened skin of the
trunk
Thickened skin of the
dorsum of the hand
12. A) Loss of finger pad substance
(B) Subcutaneous calcinosis
(C) Telangiectasias
13. Musculoskeletal disease in SSc
Flexion
contractures
Shortening of the tip of
the left index finger
Flexion contracture,
pseudoclubbing
Synovitis,
Tenosynovitis, and
Tendon Friction Rubs
14. Ischemic Digital Ulcers
Cyanotic phase of
Raynaud’s phenomenon
Pallor phase of
Raynaud’s phenomenon
Cutaneous ulcer Deep ulcer
Gangrene Autoamputation
18. Cardiac Involvement
• Range from clinically silent cardiac involvement to
frank heart failure
• Involveme of the endocardium, myocardium, and
pericardium separately or concomitantly
• Pericardial effusion
• Auricular and/or ventricular arrhythmias,
• Conduction disease
• Valvular regurgitation
• Myocardial ischemia,
• Myocardial hypertrophy
• Heart failure
19. Renal Involvement
• Scleroderma renal crisis (SRC) is a life-threatening
condition
• 5% -10% of patients with scleroderma.
• Risk factors:
early diffuse skin disease
use of corticosteroids
presence of anti-RNA polymerase III antibodies.
• Early pharmacologic intervention with ACE inhibitors is
crucial
20. Renal Involvement: may be due to
• Medication adverse effects
• Comorbid conditions
• Associated heart disease
• GI disease
• Lung disease
• Pauci-immune necrotizing crescentic glomerulonephritis
33. Natural
History of
Disease
A chronic monophasic illness
Internal organ involvement can be
predicted by the extent of skin affected
Indolent in patients with limited
scleroderma
Explosive multisystem disease can emerge
rapidly over in diffuse scleroderma
Males may have a more aggressive disease
course than do females
35. Treatment of Vascular Disease
• Organ-specific: SRC,PAH, and RP
• Nonspecific vasodilators
• Novel agents :prostacyclin, endothelin antagonists,
statins, and phosphodiesterase inhibitors
36. Treatment of Fibrosis
• To date, no drugs have proved able to reverse the fibrotic disease
process in scleroderma
• D-Penicillamine:?? Not used
• A human recombinant neutralizing TGF-β antibody:under study
• Tyrosine kinase inhibitors: Imatinib, dasatinib, and nilotinib
• Halofuginone:e ncouraging results
• Rosiglitazone
• Pirfenidone: antifibrotic and antiinflammatory properties
• Anti connective tissue growth factor (anti-CTGF)