1. PRESENTED BY:-DR. BHAGWAN DASS

2. INTRODUCTION
 Chronic episodic attacks of digital ischemia provoked
by exposure to cold or emotional stress.
 Characterized by sequential colour changes:-
 white [(pallor),> blue (cyanosis) >and red (rubor).
 Pallor is essential for diagnosis.
 Affects 10% of population, F:M=5:1
 First described by Maurice raynaud.

3.  Current terminology primary and secondary raynaud’s
phenomenon was proposed by Leroy medger
 In 1901, Jonathan Hutchinson reported that there were
several causes for the phenomenon.
 Allen and Brown established clinical criteria to
distinguish innocent primary Raynaud’s phenomenon
from secondary Raynaud’s phenomenon

4.  Classified into 2 types based on etiology;-
 Primary or idiopathic RAYNAUDS PHENOMENON
 Secondary RAYNAUDS PHENOMENON :- underlying
disease or cause is present.
 Most common cause is connective tissue disease particularly
systemic sclerosis

5. Causes of Raynaud’s phenomenon.
Primary Raynaud’s phenomenon (Raynaud’s disease)
Secondary Raynaud’s phenomenon(Raynaud's
syndrome):-
Trauma or vibration:-
 Reflex sympathetic dystrophy
 Vibration exposure
 Arteriovenous fistula
 Hypothenar hammer syndrome (ulnar artery
thrombosis)
 Intra-arterial drug administration

6.  Connective tissue disease and
vasculitis
 Systemic sclerosis
 SLE
 Rheumatoid arthritis
 Sjögren’s syndrome
 Mixed connective tissue
disease
 Dermatomyositis
 Temporal arteritis
 Hepatitis B antigen vasculitis
 Obstructive arterial disease
 Atherosclerosis
 Thromboangiitis obliterans
(TAO)(Buerger’s disease)
 Hypothenar hammer
syndrome (ulnar artery
thrombosis)
 Neurological disease
 Thoracic outlet syndrome
(cervical rib)
 Carpal tunnel syndrome
 Hypothenar hammer
syndrome
 Reflex sympathetic
dystrophy

7.  Haematological disease
 Cryoglobulinaemia
 Cold agglutinins
 Paroxysmal haemoglobinuria
 Waldenström’s
macroglobulinaemia
 Drugs and toxins
 Ergot
 β-Blockers
 Methysergide
 Bleomycin
 Amphetamines
 Imipramine
 Bromocriptine
 Clonidine
 Cyclosporin
 Oral contraceptives
 Vinyl chloride
 Nitroglycerin withdrawal
 Heavy metals
 Interferon alfa
 Miscellaneous
 Paraneoplastic syndrome
 Chronic renal failure
 Primary pulmonary
hypertension
 Hypothyroidism
 Anorexia nervosa

8. Patho- physiology
 Not fully understood but centered on a functional unit
composed of vascular endothelium, smooth muscle
cells and nerve endings.
 Exaggerated response to cold and emotional stress.
 Skin of digits supplied by both thermoregulatory and
nutritional blood flow.
 Thermoregulatory system is controlled by sympathetic
nervous system while nutritive flow is supplied by
capillary network.
 Secondary raynaud’s can lead to hypoxia and ischaemia.

9.  There are vascular defects both structural and
functional(pri. Raynaud).
 Decreased level of calcitonin gene related peptide
(CGRP) which is a potent vasodialator.
 Intravascular defects includes following:-
 Platelets activation:- increased level of thrombaxane
and serotonin.
 Defective fibrinolysis:- fibrin deposition and
obstruction of vasculature

10.  Aberrant expression of endogenous vasodilatory
substances (nitric oxide, prostacyclin, prostaglandin and
leukotrienes) and vasoconstrictors (endothelin,
angiotensin II and thromboxane A2) has been
implicated.
 WBC activation
 Decreased RBC deformability:- impairment of blood
flow.
 Oxidative stress
 Thought to be polygenic phenomenon. Recently a
genetic locus, 3p21.1-p21.3, has been identified.

11.  disturbance in vascular homeostasis may lead to
uncontrolled vasoconstriction and
 studies have demonstrated down-regulation of nitric
oxide and up-regulation of endothelin-1 in Raynaud’s
phenomenon patients.

12. Clinical features
 Age of onset:- usually under 40 years, but it may occur
over this age.
 Age and sex distribution of secondary cases is that of
underlying disease, and is not therefore so restricted to
female sex.
 Raynaud’s phenomenon affects the hands and, less
often, the feet; Changes elsewhere are exceptional,
although the nose, ear lobe, tongue etc can be
involved.

13. Loss 0f pulp 0f pad of the digit with
pitting scars and ulceration from
chronic, Severe Raynaud
phen0menon

14.  A typical attack consists of sudden pallor of one or
more digits, followed after a few minutes by cyanosis
or/and sometimes by erythema.
 Primary Raynaud’s:- symmetrical and involves several
digits.
 secondary Raynaud’s:- only one or a few digits are
affected and asymmetry is not unusual.
 Attacks are usually precipitated by cold, either local or
of the whole body, by pressure or by psychological
stimuli.

15.  Episodes may occur infrequently or many times each day.
 Severe cases:- which are usually of secondary type, may be
complicated by
 Telangiectases of the nail fold,
 Thinning and ridging of the nail, and
 Atrophy or sclerosis of the fingers (sclerodactyly).
 Skin necrosis:- rare in primary Raynaud’s phenomenon but not
uncommon in secondary Raynaud’s phenomenon and may result in
destruction of the digits.
 The disease tends to run a variable course:
 in primary Raynaud’s phenomenon the outcome is good in 80% of
cases, but some disability occurs in 20%;
 in secondary Raynaud’s phenomenon the prognosis is that of the
underlying disease.

17. Diagnosis
 Criteria for diagnosis of primary Raynaud’s phenomenon:-
1. Intermittent attacks of discoloration of extremities
2. Absence of evidence of organic peripheral arterial occlusion
3. Symmetrical or bilateral distribution
4. Exclusion of any disease, occupation, trauma or drug ingestion
that could give rise to vasospastic abnormalities
5. Absence of immunological abnormalities eg. Negative ANA
6. Female sex, age under 25 years
7. History of cold intolerance since childhood
8. Normal nail fold capillaries
9. Normal ESR
10. History of symptoms for at least 2 years.

18.  Diagnosis usually be made on basis of the history.
 Most patients describe only blanching of digits
accompanied by numbness.
 When pain is a prominent symptom in the ischemic
phase, a secondary cause should be suspected
 A careful review of systems is important to screen for
symptoms of connective tissue disease (arthralgias,
arthritis, dysphagia, heartburn, rash, photosensitiviry
skin changes, muscle weakness, shortness of breath, or
sicca),

19.  History should exclude those cases due to vibration or
continuous finger trauma and heavy metal and ergot
intoxication or any other drug.
 Buerger’s disease and other organic arterial diseases
are very rare causes of Raynaud’s phenomenon.

20.  outlet syndrome and cervical rib must be excluded in
atypical cases and where asymmetry occurs.
 Such disorders require full neurological examination,
observation of the effect on the pulse of movement at
the shoulder girdle, and radiological investigation.
 An underlying neoplasm must be sought in any case
with sudden onset in adult life, especially in men, and
if digital tip necrosis occurs.

21.  Female patients with age of onset over 25 years, no pre-
existing cold intolerance, no occupational, traumatic
or drug-related etiology, and no history of a low body
weight should be regarded as being at high risk of
developing connective tissue disease.
 The presence of abnormal capillaries on nail fold
microscopy is suggestive of a diagnosis of secondary
Raynaud’s phenomenon.

22.  In particular, Raynaud’s phenomenon(80-90%) is a
common presenting symptom of systemic sclerosis,
but if there are no other signs within 2 years of onset,
systemic sclerosis is less likely to develop.
 The presence of circulating autoantibodies
(antinuclear, anticentromere and anti-Scl 70
antibodies) should be regarded as indicating a
connective tissue disease until proven otherwise.
 A search for cryoglobulins and cold agglutinins should
be made in a patient developing Raynaud’s
phenomenon.

23. Laboratory test:-
 CBC, ESR, urinalysis,
 In Patient with ANA , specific antigen like Sm antigen or
topoisimerase or centomere or DsDNA to rule out SLE or
scleroderma
 Routine X-ray chest to look for cervical rib or evidence of
ILD.
Specific test:- Nail fold capillary microscopy procedure to
distinguish primary from secondary Raynaud phenomenon
 patients with connective tissue diseases may have enlarged,
deformed capillary loops surrounded by avascular areas.

24.  Nail fold capillary microscopy in a patient with scleroderma
showing capillary drop out with enlarged, dilated , tortuous
capillary loops

25. Approach to
a patient of
Raynaud’s
phenomenon

26. Treatment
General measures:-
 lifestyle changes to minimize exposure to cold;
 Dressing warmly with loose-fitting, layered clothing; and
keeping thermostat a few degrees higher than normal.
 Limiting time spent outdoors in winter, wearing insulated
gloves, and using hand or foot warmers are usually helpful.
 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).

27.  Patients should be strongly encouraged to stop
smoking and to avoid secondhand smoke, because
nicotine induces cutaneous vasoconstriction.
 Stress modification and social support are valuable
aspects of treatment to minimize vasoconstriction
induced by hyperactivity of sympathetic nervous
system

28. Drug therapy

29.  Calcium channel blockers:-
 in patients with primary Raynaud’s phenomenon,
decrease in attacks and reduction in symptom severity
 Recommended doses :-
 Nifedipine range from 30 to 180 mg daily.
 Amlodipine between 5 and 20 mg daily.
 Slow release or long-acting preparations are
recommended to improve compliance and reduce side
effects.

30.  Sildenafil:- phosphodiesterase inhibitor and acts by
increasing the vasodilatory effect of both nitric oxide
and prostacyclin.
 50 mg twice daily
 significant improvement in mean attack rates and
duration.
 Prostaglandins :- Intravenous infusion can reverse
ischaemic complications
 Iloprost:- a prostacyclin analogue, is commonly
administered to patients with severe digital ulceration

31.  Repeated treatments with iloprost during one year was
found to be more effective than nifedipine in reducing the
severity score of Raynaud’s phenomenon in patients with
systemic sclerosis.
 Bosentan:- an endothelin receptor antagonist(ERA), in a
study demonstratedn a marked reduction in new digital
ulcers in systemic sclerosis patients but did not decrease
the frequency or severity of Raynaud’s attacks .
 Losartan :- angiotensin II receptor antagonist
 A RCT demonstrated that it significantly reduced
frequency and severity of vasoconstrictive episodes in
patients with primary Raynaud’s phenomenon and in
Raynaud’s phenomenon secondary to systemic sclerosis.

32.  Topical glyceryl trinitrate:- signifcantly reduced the
number and severity of Raynaud’s attacks in
individuals with primary and secondary Raynaud’s
phenomenon compared with placebo;
 2% glyceryl trinitrate was associated with headaches
whereas 1% glyceryl trinitrate reduced the incidence of
side effects but maintained a similar improvement in
Raynaud’s phenomenon symptoms.

33. Sympathectomy
 Patients with refractory, disabling attacks or with an
acutely ischemic digit that is unresponsive to other
measure.
 A positive vasodilator response to a stellate ganglion
block or epidural infusion should be documented
before a permanent procedure is done

34. Management of severe vasospasm
 Severe vasospasm with prolonged ischemia (dead-
white finger) poses a threat of gangrene and
amputation; i t is considered a medical emergency.
 The patient should b e hospitalized and affected
extremity put to rest.
 Nifedipine (10 to 20 mg tid) should be started
immediately, as well as prostaglandinE 1( 6 to 10
ng/kg/minute) or PGI2, (0.5 to 2 ng/kg), given by
continuous intravenous infusion for several hours over
3 consecutive days.

35.  A digital (or stellate ganglion) block with lidocaine
hydrochloride or bupivacaine hydrochloride (without
epinephrine) relieves pain and produces a chemical
sympathectomy that may reverse vasoconstriction.
 Sympathectomy (thoracic, lumbar or digital) should
be considered in patients who have a positive response
 Arterial reconstruction should be reserved for patients
with angiographically documented occlusive vascular
disease.

36. Treatment protocol..

37. [Efficacy and safety of sildenafil in patients with systemic scleroderma].
[Article in Russian]
[No authors listed
 SUBJECTS AND RESULTS:
 Sf was used in 16 patients (including 14 women) aged 20-66 years (mean 48.6
+/- 14.6 years; median 51.5 years) with SS of a duration of 2 months to 27 years
(mean 8.8 +/- 7.3 years; median 6.5 years). The indications for Sf treatment
were significant Raynaud's phenomenon (RP) in 3 patients, digital ulcers (DU)
and/or necroses (N) in 9, pulmonary hypertension (PH) in 5 (2 patients had
PH concurrent with DU/N), and critical ischemia of the left fingers in 1 patient.
RP was seen in all the patients and so the effect of Sf on the course of RP was
evaluated in the whole patient group.
 CONCLUSION:
 Sf is an effective drug to treat the manifestations of scleroderma vasculopathy,
such as RP, DU/N, and PH. Sf is well tolerated in most cases. The SS patients
with pronounced ECG changes have an increased risk of severe cardiac events
and they need careful ECG monitoring.

38.  Raynaud phenomenon of the nipple in breastfeeding mothers: an underdiagnosed cause of nipple pain.
Barrett ME, Heller MM, Stone HF, Murase JE
 Source:-University of Southern California, Keck School of Medicine, Los Angeles, CA, USA.
 Abstract
 OBJECTIVE: To elucidate the diagnostic criteria of Raynaud phenomenon of nipple that will aid in recognizing and
treating Raynaud phenomenon in breast feeding mothers with chronic deep nipple pain during lactation.
 DESIGN: -Retrospective review of a patient database composed of 22 cases of breastfeeding mothers who fit
the diagnostic criteria for Raynaud phenomenon of the nipple.
 PATIENTS: All patients diagnosed as having Raynaud phenomenon of nipple evaluated
 MAIN OUTCOME MEASURES: The rate of failed treatment for Candida mastitis, the rate of improvement of
symptoms with nifedipine use, and the overall rate of improvement of symptoms with appropriate therapy involving
treatment of Raynaud phenomenon.
 RESULTS: Among the 22 patients with Raynaud phenomenon of the nipple, previous treatment for Candida mastitis
with oral or topical antifungals was ineffective in 20(91%). Of the 12 patients who tolerated a trial of nifedipine,10
(83%) reported decreased or resolved nipple pain. All patients experienced marked improvement of
symptoms with appropriate therapy involving treatment of Raynaud phenomenon.
 CONCLUSIONS: Most patients were treated with antifungals before presentation without resolution of nipple pain.
Nifedipine appears to be an effective medication for the treatment of Raynaud phenomenon
of the nipple. With appropriate management of Raynaud phenomenon,breastfeeding mothers demonstrated
improvement of nipple pain. Raynaud phenomenon of the nipple should be considered in the
differential diagnosis of nipple pain during lactation.

39.  Mesoglycan treatment in Raynaud phenomenon: a case series.
 Di Biase A.
 Source:-Ambulatory of Vascular Diagnostic, Geriatric Medicine Villa
Verde, Taranto, Italy - a.dibiase@email.it.
 Abstract:-
 Aim and methods: Raynaud's phenomenon (RP) is a common clinical
disorder without cure or gold standard therapy. Mesoglycan is an well-balanced
extract of glycosaminoglycans active on endothelial layers at microcirculatory
level. Herein we investigated for the first time the efficacy and tolerability of
mesoglycan on vasospastic attacks and videocapillaroscopy patterns in 25
consecutive patients with primary or secondary RP.
 Results and conclusion: During 12 months of add-on therapy, mesoglycan
obtained a remarkable and significant reduction in the frequency of Raynaud
attacks and an improvement of the capillaroscopy abnormalities in most
patients, without important adverse effects, revealing a good convenience in
the management if this condition.

40. Focal Raynaud’s phenomenon of the 5th digit related to iPad reading posture
Kathleen A. Touby1 and George C. Newman1
(1)Neurosensory Sciences, Einstein Medical Center, 5401 Old York Road, Klein 405,
Philadelphia, PA 19141, USA
 This case raises the possibility of a
compressive arteriopathy or
neuropathy resulting from a
particular reading hand posture
with the iPad.

41. THANK YOU