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 raynauds 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 Raynauds phenomenon from secondary Raynauds 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 Raynauds phenomenon. Primary Raynauds phenomenon (Raynauds disease) Secondary Raynauds 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 Sjgrens syndrome Mixed connective tissue disease Dermatomyositis Temporal arteritis Hepatitis B antigen vasculitis Obstructive arterial disease Atherosclerosis Thromboangiitis obliterans (TAO)(Buergers 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 Waldenstrms macroglobulinaemia Drugs and toxins Ergot -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 raynauds 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 Raynauds 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. Raynauds 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 Raynauds:- symmetrical and involves several digits. secondary Raynauds:- 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 Raynauds phenomenon but not uncommon in secondary Raynauds phenomenon and may result in destruction of the digits. The disease tends to run a variable course: in primary Raynauds phenomenon the outcome is good in 80% of cases, but some disability occurs in 20%; in secondary Raynauds phenomenon the prognosis is that of the underlying disease. 16. Diagnosis Criteria for diagnosis of primary Raynauds 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. 17. 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), 18. History should exclude those cases due to vibration or continuous finger trauma and heavy metal and ergot intoxication or any other drug. Buergers disease and other organic arterial diseases are very rare causes of Raynauds phenomenon. 19. 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. 20. 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 Raynauds phenomenon. 21. In particular, Raynauds 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 Raynauds phenomenon. 22. 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. 23. Nail fold capillary microscopy in a patient with scleroderma showing capillary drop out with enlarged, dilated , tortuous capillary loops 24. Approach to a patient of Raynauds phenomenon 25. 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). 26. 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 27. Drug therapy 28. Calcium channel blockers:- in patients with primary Raynauds 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. 29. 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 30. Repeated treatments with iloprost during one year was found to be more effective than nifedipine in reducing the severity score of Raynauds 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 Raynauds attacks . Losartan :- angiotensin II receptor antagonist A RCT demonstrated that it significantly reduced frequency and severity of vasoconstrictive episodes in patients with primary Raynauds phenomenon and in Raynauds phenomenon secondary to systemic sclerosis. 31. Topical glyceryl trinitrate:- signifcantly reduced the number and severity of Raynauds attacks in individuals with primary and secondary Raynauds 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 Raynauds phenomenon symptoms. 32. 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 33. 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. 34. 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. 35. Treatment protocol.. 36. [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. 37. 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. 38. 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. 39. Focal Raynauds 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. 40. THANK YOU