Raynaud’s syndrome is the most frequently encountered clinical manifestation classified as primary or secondary to underlying disease. Primary is often referred to as Raynaud’s disease, associated only with the occurrence of vasospasm alone and no other illness. But, secondary is referred to as Raynaud’s phenomenon associated with several autoimmune disease conditions. When compared to primary, the secondary has more serious consequences.
What Autoimmune Diseases are Associated with Raynaud’s?
Autoimmune disease associated with Raynaud’s is noted from the year 1950. The symptoms of the secondary usually begin after the age of 30. Progressive systemic sclerosis, systemic lupus erythematosus, Rheumatoid arthritis and Sjögren’s syndrome are some of the autoimmune diseases identified in Raynaud’s. In some individuals, this disease present in multiple forms. It is known as mixed connective tissue disease (MCTD) (or Sharp’s syndrome), which combines the feature of scleroderma, myositis, systemic lupus erythematosus, and rheumatoid arthritis. It is different from the term undifferentiated connective tissue disease (UCTD). UCTD is diagnosed in patients with existing autoimmune disease condition, but does not meet the criteria for any specific autoimmune disease.
Among the autoimmune disease associated with Raynaud’s 90% of the cases is systemic sclerosis, a connective tissue disorder. Sclerosis is characterized by the accumulation of collagen i.e., hardening and scarring of the skin. It affects the small arteries and causes the abnormal flow of the blood. There is no proper treatment for the disease and drugs for reducing the skin symptoms and inflammation are promising. Prognosis is determined by the severity of the disease form and 75% chances of survival are reported for this case.
Systematic lupus erythematosus is 10 to 44% cases found and common in Raynaud’s patients. It is an autoimmune disease affects all parts of the body including skin, joints, organs, and blood vessels. Lupus attacks the healthy immune system and treatment only available for preventing flares and decreasing severity and duration when they occur.
Sjögren syndrome 33% of cases found in Raynaud’s, which this disease affect all the moisturizing producing organ in the body. Skin, nose, mouth, eyes, vaginal dryness, chronic dry cough, joint ache, muscle ache and thyroid problems are some of the symptoms of Sjögren syndrome. Treatment meant only for reducing symptoms and no specific drug for this disease.
Rheumatoid arthritis is the initial sign of Raynaud’s disease. It is the inflammation of joints seen in hands and feet. Pains followed by stiffness are the usual symptoms in Raynaud’s. There is no therapy for this disease and however, drugs are available which change the disease form and modification has the best results. These are effective when it is managed at the earlier.
Raynaud’s phenomenon with MCTD incidence rate is higher among patients i.e., more than 85% and often this disease condition is referred as overlap syndrome. Patients have a high level of specific autoantibody and also other diseases like polymyositis, dermatomyositis, and myositis. MCTD patients complaint frequently joint pain and severe inflammation; frustration, Raynaud’s phenomenon, and hardening of the skin particularly in the fingers (sclerodactyly).
Apart from an autoimmune disease condition, Raynaud’s phenomenon also associated with numerous diseases that affect arteries. Among them, atherosclerosis was the most reported disease which causes a buildup of plaques in blood vessels of the heart. The occurrence of hypercholesterolemia in some cases was unexpectedly higher in patients with atherosclerosis underlying Raynaud’s phenomenon. Buerger’s disease, primary pulmonary hypertension, carpal tunnel syndrome, cancer, hypothyroid, acromegaly, erythromelalgia, hypersensitivity angiitis, and nerve trauma are some of the disease condition associated with Raynaud’s. Primary pulmonary hypertension is the very common condition affects the arteries of the lungs in Raynaud’s and cause significant parenchymal lung disease. Cardiac weakness and peripheral vascular spasm with pulmonary hypertension are observed in some patients in Raynaud’s phenomenon.
Raynaud’s phenomenon in people who had used repeatedly vibratory tools like drilling machine, typing occupation, and playing piano are more susceptible. The incidence rate is high in women when compared to men. Location and cold weather trigger more and hence self-care reduces the risk of the Raynaud’s attack.