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Learning Objectives
Learning Objectives
1. Introduction & History
2. Relevant Anatomy, Physiology
3. Aetiology
4. Pathophysiology
5. Pathology
6. Classification
7. Clinical Features
8. Investigations
9. Management
10. Prevention
11. Guidelines
12. Take home messages
Introduction & History.
โ€ข
Introduction
โ€ข Chronic episodic attacks of digital
ischemia provoked by exposure to cold or
emotional stress.
โ€ข Characterized by sequential colour
changes:-
โ€“ white [(pallor),> blue (cyanosis) >and red
(rubor).
Aetiology
Aetiology
โ€ข Idiopathic
โ€ข Congenital/ Genetic
โ€ข Nutritional Deficiency/excess
โ€ข Traumatic
โ€ข Infections /Infestation
โ€ข Autoimmune
โ€ข Neoplastic (Benign/Malignant)
โ€ข Degenerative / lifestyle
โ€ข Iatrogenic
โ€ข Psychosomatic
โ€ข Poisoning/ Toxins/ Drug induced
Etiology
โ€ข Idiopathic
โ€ข Congenital
โ€ข Traumatic
โ€ข Infections /Infestation
โ€ข Autoimmune
โ€ข Neoplastic (Benign/Malignant)
โ€ข Degenerative
Etiology
โ€“ Primary or idiopathic RAYNAUDS PHENOMENON
โ€“ Secondary RAYNAUDS PHENOMENON :- underlying
disease or cause is present.
โ€ข Most common cause is connective tissue disease
particularly systemic sclerosis
Etiology:Primary Raynaudโ€™s
phenomenon (Raynaudโ€™s disease)
๏ƒ˜Trauma or vibration:-
โ€“ Reflex sympathetic dystrophy
โ€“ Vibration exposure
โ€“ Arteriovenous fistula
โ€“ Hypothenar hammer syndrome (ulnar artery
thrombosis)
โ€“ Intra-arterial drug administration
Etiology:Primary Raynaudโ€™s
phenomenon (Raynaudโ€™s disease)
๏ƒ˜ Connective tissue disease
and vasculitis
โ€“ Systemic sclerosis
โ€“ SLE
โ€“ Rheumatoid arthritis
โ€“ Sjรถgrenโ€™s syndrome
โ€“ Mixed connective tissue
disease
โ€“ Dermatomyositis
โ€“ Temporal arteritis
โ€“ Hepatitis B antigen vasculitis
๏ƒ˜ Obstructive arterial disease
โ€“ Atherosclerosis
โ€“ Thromboangiitis obliterans
(TAO)(Buergerโ€™s disease)
โ€“ Hypothenar hammer
syndrome (ulnar artery
thrombosis)
๏ƒ˜ Neurological disease
โ€“ Thoracic outlet syndrome
(cervical rib)
โ€“ Carpal tunnel syndrome
โ€“ Hypothenar hammer
syndrome
โ€“ Reflex sympathetic
dystrophy
Etiology:Primary Raynaudโ€™s
phenomenon (Raynaudโ€™s disease)
๏ƒ˜ Haematological disease
โ€“ Cryoglobulinaemia
โ€“ Cold agglutinins
โ€“ Paroxysmal haemoglobinuria
โ€“ Waldenstrรถmโ€™s
macroglobulinaemia
๏ƒ˜ Drugs and toxins
โ€“ Ergot
โ€“ ฮฒ-Blockers
โ€“ Methysergide
โ€“ Bleomycin
โ€“ Amphetamines
โ€“ Imipramine
โ€“ Bromocriptine
โ€“ Clonidine
โ€“ Cyclosporin
โ€“ Oral contraceptives
โ€“ Vinyl chloride
โ€“ Nitroglycerin withdrawal
โ€“ Heavy metals
โ€“ Interferon alfa
๏ƒ˜ Miscellaneous
โ€“ Paraneoplastic syndrome
โ€“ Chronic renal failure
โ€“ Primary pulmonary hypertension
โ€“ Hypothyroidism
โ€“ Anorexia nervosa
Pathophysiology
Pathophysiology
โ€ข Not fully understood
โ€ข Exaggerated response to cold and emotional
stress.
โ€ข disturbance in vascular homeostasis leads to
uncontrolled vasoconstriction and
โ€ข studies have demonstrated down-regulation
of nitric oxide and up-regulation of
endothelin-1 in Raynaudโ€™s phenomenon
patients.
Clinical Features
โ€ข
Clinical Features
โ€ข Demography
โ€ข Symptoms
โ€ข Signs
โ€ข Prognosis
โ€ข Complications
Demography
Demography
โ€ข Age of onset:- usually under 40 years, but it
may occur over this age.
โ€ข Age and sex distribution of secondary cases
is that of underlying disease, and is not
therefore so restricted to female sex.
โ€ข Raynaudโ€™s phenomenon affects the hands
and, less often, the feet; Changes elsewhere
are exceptional, although the nose, ear lobe,
tongue etc can be involved
Symptoms
Symptoms & Signs
โ€ข A typical attack consists of sudden pallor of
one or more digits, followed after a few
minutes by cyanosis or/and sometimes by
erythema.
โ€ข Attacks are usually precipitated by cold,
either local or of the whole body, by
pressure or by psychological stimuli
Prognosis
Prognosis
โ€ข Morbidity
โ€ข Mortality rate
โ€ข 5 year survival in Malignancy
Prognosis
โ€ข The disease tends to run a variable course:
โ€“ in primary Raynaudโ€™s phenomenon the
outcome is good in 80% of cases, but some
disability occurs in 20%;
โ€“ in secondary Raynaudโ€™s phenomenon the
prognosis is that of the underlying
Complications
Complications
โ€“ Telangiectases of the nail fold,
โ€“ Thinning and ridging of the nail, and
โ€“ Atrophy or sclerosis of the fingers
(sclerodactyly).
โ€“ Skin necrosis:- rare in primary Raynaudโ€™s
phenomenon but not uncommon in secondary
Raynaudโ€™s phenomenon and may result in
destruction of the digits.
Investigations
Investigations
โ€ข Laboratory Studies
โ€“ Routine
โ€“ Special
โ€ข Imaging Studies
โ€ข Tissue diagnosis
โ€“ Cytology
โ€ข FNAC
โ€“ Histology
โ€“ Germ line Testing and Molecular Analysis
โ€ข Diagnostic Laparotomy.
Investigations
โ€ข Laboratory Studies
โ€“ Routine
โ€“ Special
โ€ข Imaging Studies
โ€ข Tissue diagnosis
โ€“ Cytology
โ€ข FNAC
โ€“ Histlogy
Laboratory Studies
Laboratory Studies
โ€ข CBC, ESR, urinalysis,
โ€ข In Patient with ANA , specific antigen like
Sm antigen or topoisimerase or centomere
or DsDNA to rule out SLE or scleroderma
Diagnostic Studies
Imaging Studies
Diagnostic Studies
Imaging Studies
โ€ข X-Ray
โ€ข USG
โ€ข CT
โ€ข Angiography
โ€ข MRI
โ€ข Endoscopy
โ€ข Nuclear scan
Diagnostic Studies
Imaging Studies
โ€ข X-RayRoutine X-ray chest to look for
cervical rib or evidence of ILD.
Management
Management
โ€ข lifestyle changes to minimize exposure to
cold;
โ€ข Dressing warmly with loose-fitting, layered
clothing; and keeping thermostat a few
degrees higher than normal.
โ€ข Limiting time spent outdoors in winter,
wearing insulated gloves, and using hand or
foot warmers are usually helpful.
Management
โ€ข Patients should be taught to recognize and
terminate attacks promptly by returning to a
warmer environment and applying local
heat to hands (..g., by placing their hands in
warm water or by using a hair dryer).
โ€ข Stop smoking and to avoid secondhand
smoke,
โ€ข Stress modification and social support to
minimize vasoconstriction induced by
hyperactivity of sympathetic nervous
system
Non Operative Therapy
Non Operative Therapy
โ€ข Calcium channel blockers
โ€ข Sildenafil
โ€ข Prostaglandins
โ€ข Iloprost:- a prostacyclin analogue
โ€ข Bosentan:- an endothelin receptor
antagonist(ERA)
โ€ข Losartan :- angiotensin II receptor
antagonist
โ€ข Topical glyceryl trinitrate
Questions
โ€ข Full question
โ€ข Short Note
โ€ข Long case
โ€ข Short case
โ€ข MCQs
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Raynaud's phenomenon.pptx

  • 1. Tips on using my ppt. 1. You can freely download, edit, modify and put your name etc. 2. Donโ€™t be concerned about number of slides. Half the slides are blanks except for the title. 3. First show the blank slides (eg. Aetiology ) > Ask students what they already know about ethology of today's topic. > Then show next slide which enumerates aetiologies. 4. At the end rerun the show โ€“ show blank> ask questions > show next slide. 5. This will be an ACTIVE LEARNING SESSION x three revisions. 6. Good for self study also. 7. See notes for bibliography.
  • 3. Learning Objectives 1. Introduction & History 2. Relevant Anatomy, Physiology 3. Aetiology 4. Pathophysiology 5. Pathology 6. Classification 7. Clinical Features 8. Investigations 9. Management 10. Prevention 11. Guidelines 12. Take home messages
  • 5. Introduction โ€ข Chronic episodic attacks of digital ischemia provoked by exposure to cold or emotional stress. โ€ข Characterized by sequential colour changes:- โ€“ white [(pallor),> blue (cyanosis) >and red (rubor).
  • 7. Aetiology โ€ข Idiopathic โ€ข Congenital/ Genetic โ€ข Nutritional Deficiency/excess โ€ข Traumatic โ€ข Infections /Infestation โ€ข Autoimmune โ€ข Neoplastic (Benign/Malignant) โ€ข Degenerative / lifestyle โ€ข Iatrogenic โ€ข Psychosomatic โ€ข Poisoning/ Toxins/ Drug induced
  • 8. Etiology โ€ข Idiopathic โ€ข Congenital โ€ข Traumatic โ€ข Infections /Infestation โ€ข Autoimmune โ€ข Neoplastic (Benign/Malignant) โ€ข Degenerative
  • 9. Etiology โ€“ Primary or idiopathic RAYNAUDS PHENOMENON โ€“ Secondary RAYNAUDS PHENOMENON :- underlying disease or cause is present. โ€ข Most common cause is connective tissue disease particularly systemic sclerosis
  • 10. Etiology:Primary Raynaudโ€™s phenomenon (Raynaudโ€™s disease) ๏ƒ˜Trauma or vibration:- โ€“ Reflex sympathetic dystrophy โ€“ Vibration exposure โ€“ Arteriovenous fistula โ€“ Hypothenar hammer syndrome (ulnar artery thrombosis) โ€“ Intra-arterial drug administration
  • 11. Etiology:Primary Raynaudโ€™s phenomenon (Raynaudโ€™s disease) ๏ƒ˜ Connective tissue disease and vasculitis โ€“ Systemic sclerosis โ€“ SLE โ€“ Rheumatoid arthritis โ€“ Sjรถgrenโ€™s syndrome โ€“ Mixed connective tissue disease โ€“ Dermatomyositis โ€“ Temporal arteritis โ€“ Hepatitis B antigen vasculitis ๏ƒ˜ Obstructive arterial disease โ€“ Atherosclerosis โ€“ Thromboangiitis obliterans (TAO)(Buergerโ€™s disease) โ€“ Hypothenar hammer syndrome (ulnar artery thrombosis) ๏ƒ˜ Neurological disease โ€“ Thoracic outlet syndrome (cervical rib) โ€“ Carpal tunnel syndrome โ€“ Hypothenar hammer syndrome โ€“ Reflex sympathetic dystrophy
  • 12. Etiology:Primary Raynaudโ€™s phenomenon (Raynaudโ€™s disease) ๏ƒ˜ Haematological disease โ€“ Cryoglobulinaemia โ€“ Cold agglutinins โ€“ Paroxysmal haemoglobinuria โ€“ Waldenstrรถmโ€™s macroglobulinaemia ๏ƒ˜ Drugs and toxins โ€“ Ergot โ€“ ฮฒ-Blockers โ€“ Methysergide โ€“ Bleomycin โ€“ Amphetamines โ€“ Imipramine โ€“ Bromocriptine โ€“ Clonidine โ€“ Cyclosporin โ€“ Oral contraceptives โ€“ Vinyl chloride โ€“ Nitroglycerin withdrawal โ€“ Heavy metals โ€“ Interferon alfa ๏ƒ˜ Miscellaneous โ€“ Paraneoplastic syndrome โ€“ Chronic renal failure โ€“ Primary pulmonary hypertension โ€“ Hypothyroidism โ€“ Anorexia nervosa
  • 14. Pathophysiology โ€ข Not fully understood โ€ข Exaggerated response to cold and emotional stress. โ€ข disturbance in vascular homeostasis leads to uncontrolled vasoconstriction and โ€ข studies have demonstrated down-regulation of nitric oxide and up-regulation of endothelin-1 in Raynaudโ€™s phenomenon patients.
  • 16. Clinical Features โ€ข Demography โ€ข Symptoms โ€ข Signs โ€ข Prognosis โ€ข Complications
  • 18. Demography โ€ข Age of onset:- usually under 40 years, but it may occur over this age. โ€ข Age and sex distribution of secondary cases is that of underlying disease, and is not therefore so restricted to female sex. โ€ข Raynaudโ€™s phenomenon affects the hands and, less often, the feet; Changes elsewhere are exceptional, although the nose, ear lobe, tongue etc can be involved
  • 20. Symptoms & Signs โ€ข A typical attack consists of sudden pallor of one or more digits, followed after a few minutes by cyanosis or/and sometimes by erythema. โ€ข Attacks are usually precipitated by cold, either local or of the whole body, by pressure or by psychological stimuli
  • 22. Prognosis โ€ข Morbidity โ€ข Mortality rate โ€ข 5 year survival in Malignancy
  • 23. Prognosis โ€ข The disease tends to run a variable course: โ€“ in primary Raynaudโ€™s phenomenon the outcome is good in 80% of cases, but some disability occurs in 20%; โ€“ in secondary Raynaudโ€™s phenomenon the prognosis is that of the underlying
  • 25. Complications โ€“ Telangiectases of the nail fold, โ€“ Thinning and ridging of the nail, and โ€“ Atrophy or sclerosis of the fingers (sclerodactyly). โ€“ Skin necrosis:- rare in primary Raynaudโ€™s phenomenon but not uncommon in secondary Raynaudโ€™s phenomenon and may result in destruction of the digits.
  • 27. Investigations โ€ข Laboratory Studies โ€“ Routine โ€“ Special โ€ข Imaging Studies โ€ข Tissue diagnosis โ€“ Cytology โ€ข FNAC โ€“ Histology โ€“ Germ line Testing and Molecular Analysis โ€ข Diagnostic Laparotomy.
  • 28. Investigations โ€ข Laboratory Studies โ€“ Routine โ€“ Special โ€ข Imaging Studies โ€ข Tissue diagnosis โ€“ Cytology โ€ข FNAC โ€“ Histlogy
  • 30. Laboratory Studies โ€ข CBC, ESR, urinalysis, โ€ข In Patient with ANA , specific antigen like Sm antigen or topoisimerase or centomere or DsDNA to rule out SLE or scleroderma
  • 32. Diagnostic Studies Imaging Studies โ€ข X-Ray โ€ข USG โ€ข CT โ€ข Angiography โ€ข MRI โ€ข Endoscopy โ€ข Nuclear scan
  • 33. Diagnostic Studies Imaging Studies โ€ข X-RayRoutine X-ray chest to look for cervical rib or evidence of ILD.
  • 35. Management โ€ข lifestyle changes to minimize exposure to cold; โ€ข Dressing warmly with loose-fitting, layered clothing; and keeping thermostat a few degrees higher than normal. โ€ข Limiting time spent outdoors in winter, wearing insulated gloves, and using hand or foot warmers are usually helpful.
  • 36. Management โ€ข Patients should be taught to recognize and terminate attacks promptly by returning to a warmer environment and applying local heat to hands (..g., by placing their hands in warm water or by using a hair dryer). โ€ข Stop smoking and to avoid secondhand smoke, โ€ข Stress modification and social support to minimize vasoconstriction induced by hyperactivity of sympathetic nervous system
  • 38. Non Operative Therapy โ€ข Calcium channel blockers โ€ข Sildenafil โ€ข Prostaglandins โ€ข Iloprost:- a prostacyclin analogue โ€ข Bosentan:- an endothelin receptor antagonist(ERA) โ€ข Losartan :- angiotensin II receptor antagonist โ€ข Topical glyceryl trinitrate
  • 39. Questions โ€ข Full question โ€ข Short Note โ€ข Long case โ€ข Short case โ€ข MCQs
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Editor's Notes

  1. drpradeeppande@gmail.com 7697305442