Raynaud’s phenomenon is a condition that causes some areas of the body, such as the fingers, toes, tip of the nose and ears, to feel numb and cool in response to cold temperatures or stress. This is called a Raynaud’s attack and it results from a temporary reduction in blood flow to those areas. Raynaud’s phenomenon can occur on its own or secondary to an underlying autoimmune disease. Some of the autoimmune diseases that are associated with secondary Raynaud’s phenomenon include:
Scleroderma
Scleroderma is an autoimmune rheumatic disease that leads to hardening and tightening of the skin and connective tissues. It is characterized by fibrosis (scarring) and vascular alterations. There are two main types of scleroderma:
- Localized scleroderma – This affects only the skin and involves circumscribed patches or bands of hardened skin. It does not affect internal organs.
- Systemic scleroderma or systemic sclerosis – This type affects the skin as well as internal organs such as the gastrointestinal tract, lungs, heart and kidneys. It is further classified into limited cutaneous systemic sclerosis and diffuse cutaneous systemic sclerosis based on the extent of skin involvement.
About 90% of people with systemic scleroderma experience Raynaud’s phenomenon. It is typically one of the early manifestations of the disease, presenting before skin changes become evident. The underlying blood vessel abnormalities in scleroderma predispose these patients to exaggerated vasospastic responses to cold exposure or stress, resulting in Raynaud’s attacks.
Treatment
Treatment for Raynaud’s phenomenon secondary to scleroderma focuses on managing symptoms and preventing complications like digital ulcers. Lifestyle measures include avoiding cold temperatures, smoking cessation, keeping warm with gloves and socks, and managing stress. Medications that can help dilate blood vessels include calcium channel blockers, ACE inhibitors, prostacyclins and phosphodiesterase type-5 inhibitors. More severe cases may require intravenous prostanoids.
Rheumatoid Arthritis
Rheumatoid arthritis (RA) is an inflammatory autoimmune disease characterized by joint inflammation and damage. It typically affects the small joints of the hands and feet in a symmetrical pattern. RA causes swelling, stiffness and pain in the affected joints.
About 20% of people with RA experience Raynaud’s phenomenon. The underlying autoimmunity and inflammation in RA can affect blood vessels, making them overreact to cold or stress. Women are more commonly affected than men.
Treatment
As with scleroderma, treatment focuses on relieving Raynaud’s symptoms and preventing complications. Lifestyle measures, quitting smoking, keeping warm, avoiding cold exposure, and managing stress can help reduce frequency and severity of attacks. Medications like calcium channel blockers and topical nitrates may be used. Severe cases may require immune-modulating drugs.
Sjögren’s Syndrome
Sjögren’s syndrome is an autoimmune condition affecting the exocrine glands, mainly the salivary and tear glands. This causes dryness of the mouth and eyes. Sjögren’s can occur by itself (primary form) or alongside other autoimmune diseases like rheumatoid arthritis (secondary form).
About 10-30% of Sjögren’s patients experience Raynaud’s phenomenon. The underlying autoimmune inflammation can damage blood vessels and nerves controlling vascular tone. Women are 9 times more likely to develop Sjögren’s syndrome compared to men.
Treatment
Artificial tears and saliva substitutes help manage dry eyes and dry mouth symptoms. Medications like pilocarpine and cevimeline may improve saliva production. For Raynaud’s phenomenon, lifestyle measures and medications like calcium channel blockers can help reduce frequency and severity of attacks. Immunosuppressants like methotrexate may be used in severe cases.
Mixed Connective Tissue Disease
Mixed connective tissue disease (MCTD) is characterized by overlapping features of 3 autoimmune diseases – systemic sclerosis, polymyositis/dermatomyositis, systemic lupus erythematosus and rheumatoid arthritis. Raynaud’s phenomenon is one of the most common manifestations, seen in over 95% of patients with MCTD.
Widespread vascular damage from chronic inflammation predisposes these patients to exaggerated narrowing of blood vessels in response to cold or stress. Women are more commonly affected than men.
Treatment
Low dose corticosteroids and immunosuppressive drugs like methotrexate help manage inflammation and autoimmune activity in MCTD. Calcium channel blockers, Losartan and PDE-5 inhibitors help treat Raynaud’s phenomenon. Lifestyle measures also play an important role.
Systemic Lupus Erythematosus
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can affect any organ system. It causes widespread inflammation and damage to tissues and blood vessels. Raynaud’s phenomenon occurs in about 10-45% of people with SLE.
Blood vessel damage from chronic inflammation as well as certain antibodies predispose SLE patients to exaggerated vascular spasm and Raynaud’s attacks. Women are 9 times more likely to have SLE compared to men.
Treatment
Antimalarial drugs like hydroxychloroquine and immunosuppressants like methotrexate and azathioprine help control inflammation and autoimmune activity in SLE. Raynaud’s phenomenon is treated with calcium channel blockers, Losartan, PDE-5 inhibitors and lifestyle measures.
Undifferentiated Connective Tissue Disease
Undifferentiated connective tissue disease (UCTD) refers to a condition where people have signs and symptoms suggestive of a systemic autoimmune disease like lupus, scleroderma or rheumatoid arthritis but do not meet the diagnostic criteria for a definitive diagnosis.
About 40-70% of people with UCTD experience Raynaud’s phenomenon. The underlying autoimmune inflammation can damage blood vessels and nerves controlling vascular tone, predisposing them to exaggerated vasoconstriction.
Treatment
Low dose corticosteroids and immunosuppressive medications like methotrexate help control inflammation in UCTD. Raynaud’s phenomenon is managed with lifestyle measures and medications like calcium channel blockers, Losartan and phosphodiesterase inhibitors.
Polymyositis and Dermatomyositis
Polymyositis and dermatomyositis are inflammatory myopathies characterized by muscle weakness and rashes. They are caused by autoimmune destruction of muscle fibers. Up to 40% of patients experience Raynaud’s phenomenon.
Chronic inflammation damages blood vessels, causing them to overreact to cold exposure or stress. Dermatomyositis rashes typically precede muscle weakness while Raynaud’s attacks tend to occur after onset of muscle involvement.
Treatment
Corticosteroids, intravenous immunoglobulin and immunosuppressive medications like methotrexate and azathioprine help suppress autoimmune activity and inflammation. Raynaud’s phenomenon is managed with lifestyle measures, calcium channel blockers and phosphodiesterase inhibitors.
Primary Biliary Cholangitis
Primary biliary cholangitis (PBC) is an autoimmune disease characterized by progressive destruction of the bile ducts in the liver. This impairs bile flow and damages liver tissue. About 25-45% of patients with PBC experience Raynaud’s phenomenon.
Chronic inflammation in PBC can damage blood vessels and cause vascular hyperreactivity. Antimitochondrial antibodies may also play a role. Women are 9 times more likely to develop PBC compared to men.
Treatment
Ursodeoxycholic acid helps slow progression of liver damage in PBC. Immunosuppressants like methotrexate may be used to control inflammation and autoimmunity. Raynaud’s phenomenon is treated with lifestyle measures, calcium channel blockers and vascular dilators.
Autoimmune Hepatitis
Autoimmune hepatitis is characterized by chronic inflammation and damage to liver cells due to autoimmune destruction. About 13-20% of patients experience Raynaud’s phenomenon.
Chronic autoimmune-mediated inflammation is thought to damage blood vessels and make them hyperreactive. Women are more commonly affected than men.
Treatment
Corticosteroids and azathioprine help suppress autoimmune destruction of the liver. Raynaud’s phenomenon is managed through lifestyle measures and medications like nifedipine, amlodipine, nitrates and phosphodiesterase inhibitors.
Conclusion
Raynaud’s phenomenon can occur as a primary condition or secondary to a number of autoimmune diseases. Some of the autoimmune diseases closely associated with secondary Raynaud’s include scleroderma, rheumatoid arthritis, Sjögren’s syndrome, mixed connective tissue disease, systemic lupus erythematosus, polymyositis/dermatomyositis, primary biliary cholangitis and autoimmune hepatitis.
Chronic inflammation from autoimmune conditions can damage blood vessels and nerves that control vascular tone. This makes the blood vessels overreact to cold temperatures or stress, leading to exaggerated vasoconstriction and reduced blood flow i.e. Raynaud’s attacks. Lifestyle measures and medications that relax blood vessels can help manage symptoms.
Raynaud’s phenomenon is more common in women, likely related to hormonal factors. Prompt diagnosis and treatment of the underlying autoimmune disease is key to prevent complications like digital ulcers and optimize quality of life.