Rowell’s syndrome is a rare autoimmune disorder that is characterized by the presence of three major symptoms: subacute cutaneous lupus erythematosus (SCLE), rheumatoid arthritis (RA), and positive anti-SSA/Ro antibodies. It was first described by Rowell in 1963 and is considered to be a variant of lupus erythematosus. In this article, we will discuss the definition and types of Rowell’s syndrome and provide a detailed explanation of the condition.
Definition of Rowell’s Syndrome
Rowell’s syndrome is an autoimmune disorder that affects the skin, joints, and other organs in the body. The syndrome is characterized by the presence of three major symptoms, which are:
- Subacute cutaneous lupus erythematosus (SCLE): SCLE is a type of lupus erythematosus that affects the skin. It is characterized by the presence of scaly, erythematous (red) patches on the skin that are usually located on sun-exposed areas of the body. The lesions are typically annular (ring-shaped) or psoriasiform (scaly) and may be accompanied by mild itching or burning.
- Rheumatoid arthritis (RA): RA is a chronic autoimmune disorder that affects the joints, causing pain, swelling, and stiffness. The joints most commonly affected by RA are those in the hands, feet, and wrists. Other symptoms of RA may include fatigue, fever, and weight loss.
- Positive anti-SSA/Ro antibodies: Anti-SSA/Ro antibodies are autoantibodies that are produced by the immune system in response to the presence of an antigen. In Rowell’s syndrome, these antibodies are present in the blood of affected individuals and are associated with the development of SCLE.
Types of Rowell’s Syndrome
There are two types of Rowell’s syndrome, which are based on the order of appearance of the symptoms:
- Classic type: In this type, the initial symptoms are SCLE and positive anti-SSA/Ro antibodies, which are later followed by the development of RA. This type of Rowell’s syndrome is more common than the second type.
- Inverse type: In this type, the initial symptoms are RA, which is later followed by the development of SCLE and positive anti-SSA/Ro antibodies. This type of Rowell’s syndrome is rare.
Causes
The exact cause of Rowell’s syndrome is still unknown. However, there are several factors that have been suggested to be involved in the development of this disease and possible causes for Rowell’s syndrome and explain their details.
- Genetics: Genetics is believed to play a significant role in the development of Rowell’s syndrome. Studies have shown that there is an increased risk of developing this disease if a family member has it. Specific genes have been identified that may be involved in the development of this disease.
- Environmental Factors: Environmental factors such as exposure to sunlight, medications, chemicals, and infections have been linked to the development of Rowell’s syndrome.
- Viral Infections: Viral infections such as herpes simplex virus, Epstein-Barr virus, cytomegalovirus, and human immunodeficiency virus (HIV) have been associated with the development of Rowell’s syndrome.
- Bacterial Infections: Bacterial infections such as Streptococcus, Mycoplasma pneumonia, and Helicobacter pylori have been linked to the development of Rowell’s syndrome.
- Fungal Infections: Fungal infections such as candidiasis have been linked to the development of Rowell’s syndrome.
- Drug Reactions: Certain medications have been associated with the development of Rowell’s syndrome. Examples include sulfonamides, penicillin, and non-steroidal anti-inflammatory drugs (NSAIDs).
- Lupus: Individuals who already have lupus are at a higher risk of developing Rowell’s syndrome.
- Gender: Women are more likely to develop Rowell’s syndrome than men.
- Age: Rowell’s syndrome is more common in individuals between the ages of 40 and 60.
- Hormones: Hormonal imbalances have been associated with the development of Rowell’s syndrome.
- Sunlight: Exposure to sunlight has been linked to the development of Rowell’s syndrome.
- Stress: Stress can weaken the immune system, making it more susceptible to autoimmune diseases such as Rowell’s syndrome.
- Smoking: Smoking has been associated with the development of Rowell’s syndrome.
- Alcohol: Alcohol consumption has been linked to the development of Rowell’s syndrome.
- Nutritional Deficiencies: Nutritional deficiencies, such as vitamin D deficiency, have been associated with the development of Rowell’s syndrome.
- Autoimmune Diseases: Individuals who have other autoimmune diseases are at a higher risk of developing Rowell’s syndrome.
- Abnormal Immune System Response: Abnormal immune system response can trigger the development of Rowell’s syndrome.
- Radiation Exposure: Exposure to radiation has been linked to the development of Rowell’s syndrome.
- Allergies: Allergies can weaken the immune system, making it more susceptible to autoimmune diseases such as Rowell’s syndrome.
- Unknown Causes: In some cases, the cause of Rowell’s syndrome is unknown.
Symptoms
Symptoms of Rowell’s syndrome in detail.
- Skin rash: A red, scaly rash may appear on the face, neck, chest, back, or other parts of the body. The rash is often the first symptom of Rowell’s syndrome, and it may be itchy or painful.
- Joint pain and stiffness: Patients with Rowell’s syndrome may experience joint pain and stiffness, similar to those with rheumatoid arthritis. The joints most commonly affected are the hands, wrists, knees, and ankles.
- Muscle weakness: This is a common symptom of dermatomyositis, which is one of the autoimmune diseases associated with Rowell’s syndrome. The weakness may affect the muscles of the arms, legs, and torso.
- Raynaud’s phenomenon: This is a condition in which the fingers and toes turn white or blue in response to cold temperatures or emotional stress. It is caused by the narrowing of blood vessels in the extremities, and it is common in patients with SLE.
- Photosensitivity: Patients with Rowell’s syndrome may be sensitive to sunlight, which can cause a rash or other skin reaction.
- Mouth ulcers: These are painful sores that can appear on the inside of the mouth or lips. They may be a sign of SLE or another autoimmune disease.
- Hair loss: This is a common symptom of SLE, and it may occur in patches or throughout the scalp.
- Fatigue: Patients with Rowell’s syndrome may feel extremely tired and have difficulty performing daily tasks.
- Weight loss: Unintentional weight loss may occur in patients with Rowell’s syndrome, especially in those with severe symptoms.
- Fever: Patients with Rowell’s syndrome may experience fever, especially during disease flares.
- Dry eyes and mouth: This is a common symptom of Sjogren’s syndrome, which is another autoimmune disease that can occur with Rowell’s syndrome.
- Difficulty swallowing: Patients with Rowell’s syndrome may have difficulty swallowing food or liquids, which can be a sign of muscle weakness or inflammation.
- Chest pain: This can occur if Rowell’s syndrome affects the heart or lungs.
- Shortness of breath: This can also occur if the lungs are affected by Rowell’s syndrome.
- Abdominal pain: This may occur if Rowell’s syndrome affects the digestive system, causing inflammation or other problems.
- Kidney problems: Patients with Rowell’s syndrome may develop kidney problems, which can cause swelling, high blood pressure, and other symptoms.
- Headaches: Patients with SLE may experience frequent headaches, which can be severe.
- Neuropathy: This is a condition in which the nerves that carry messages to and from the brain and spinal cord are damaged. It can cause numbness, tingling, and weakness in the limbs.
- Seizures: In rare cases, patients with Rowell’s syndrome may experience seizures, which are caused by abnormal electrical activity in the brain.
- Cognitive dysfunction: Patients with SLE may experience cognitive dysfunction, which can cause problems with memory
Diagnosis
Common diagnostic tests and procedures used to diagnose Rowell’s syndrome.
- Medical History: A detailed medical history of the patient is taken to look for any past medical conditions or family history of autoimmune diseases.
- Physical examination: A comprehensive physical examination is done to look for any skin rashes, joint inflammation, or other symptoms of autoimmune diseases.
- Blood tests: Blood tests are performed to check the levels of different antibodies in the blood, including anti-Ro/La antibodies, rheumatoid factor, antinuclear antibodies (ANA), and complement levels.
- Skin biopsy: A skin biopsy is a procedure in which a small piece of skin is removed for examination under a microscope. This is done to check for any lupus erythematosus-like skin lesions.
- Joint aspiration: Joint aspiration involves removing a sample of synovial fluid from an inflamed joint. This is done to check for any signs of rheumatoid arthritis.
- X-rays: X-rays are used to look for any signs of joint damage, which can be indicative of rheumatoid arthritis.
- Ultrasound: Ultrasound is a non-invasive imaging test that can be used to examine the joints and look for signs of inflammation.
- Magnetic resonance imaging (MRI): MRI uses powerful magnets and radio waves to produce detailed images of the body. It can be used to examine the joints and internal organs.
- Computed tomography (CT) scan: CT scans use X-rays and computer technology to produce detailed images of the body. They can be used to examine the internal organs.
- Electrocardiogram (ECG): An ECG is a test that measures the electrical activity of the heart. It can be used to check for any abnormalities in the heart.
- Echocardiogram: An echocardiogram is a non-invasive test that uses sound waves to produce images of the heart. It can be used to look for any abnormalities in the heart.
- Pulmonary function tests: These are a series of tests that measure how well the lungs are functioning. They can be used to check for any lung involvement in Rowell’s syndrome.
- Urinalysis: Urinalysis involves testing a sample of urine for the presence of protein and other substances. This can help to identify any kidney involvement in Rowell’s syndrome.
- Renal function tests: These are a series of blood tests that are used to check how well the kidneys are functioning.
- Liver function tests: These are a series of blood tests that are used to check how well the liver is functioning.
- Stool analysis: A stool analysis is done to look for any signs of inflammation in the digestive system.
- Endoscopy: Endoscopy involves using a thin, flexible tube with a camera to examine the digestive system. This is done to look for any signs of inflammation or damage.
- Colonoscopy: A colonoscopy is a type of endoscopy that is used to examine the colon and rectum.
- Chest X-ray: A chest X-ray is used to look for any abnormalities in the lungs or chest.
- Serologic tests: Serologic tests involve testing a sample of blood for the presence of specific antibodies. These can be used to diagnose certain autoimmune diseases, including Rowell’s syndrome.
Treatment
Treatments for Rowell’s syndrome, including medications, therapies, and lifestyle changes.
- Nonsteroidal anti-inflammatory drugs (NSAIDs): NSAIDs are used to reduce inflammation, pain, and fever associated with Rowell’s syndrome. They work by blocking the production of prostaglandins, which are chemicals that contribute to inflammation.
- Corticosteroids: Corticosteroids are powerful anti-inflammatory drugs that are used to treat the symptoms of Rowell’s syndrome. They work by reducing inflammation and suppressing the immune system.
- Antimalarials: Antimalarials are a group of medications that are commonly used to treat SLE. They have been shown to be effective in treating the skin and joint symptoms of Rowell’s syndrome.
- Immunomodulators: Immunomodulators are medications that work by modifying the activity of the immune system. They are used to treat a variety of autoimmune disorders, including Rowell’s syndrome.
- Methotrexate: Methotrexate is a medication that is commonly used to treat RA. It works by inhibiting the production of cells that contribute to inflammation.
- Cyclophosphamide: Cyclophosphamide is a chemotherapy drug that is used to treat autoimmune disorders. It works by suppressing the immune system.
- Azathioprine: Azathioprine is an immunosuppressive medication that is commonly used to treat autoimmune disorders. It works by suppressing the activity of the immune system.
- Mycophenolate mofetil: Mycophenolate mofetil is an immunosuppressive medication that is used to treat autoimmune disorders. It works by suppressing the activity of the immune system.
- Belimumab: Belimumab is a medication that is used to treat SLE. It works by targeting a protein that contributes to the abnormal activity of the immune system.
- Rituximab: Rituximab is a medication that is used to treat autoimmune disorders. It works by targeting specific cells in the immune system that contribute to inflammation.
- Plasmapheresis: Plasmapheresis is a procedure that is used to remove antibodies from the blood. It is used to treat severe cases of Rowell’s syndrome.
- Intravenous immunoglobulin (IVIG): IVIG is a treatment that involves giving a person immunoglobulin through a vein. It is used to treat a variety of autoimmune disorders, including Rowell’s syndrome.
- Ultraviolet (UV) light therapy: UV light therapy is a type of phototherapy that involves exposing the skin to UV light. It is used to treat the skin symptoms of Rowell’s syndrome.
- Topical corticosteroids: Topical corticosteroids are creams or ointments that are applied directly to the skin. They are used to reduce inflammation and itching associated with Rowell’s syndrome.
- Topical calcineurin inhibitors: Topical calcineurin inhibitors are medications that are used to treat the skin symptoms of Rowell’s syndrome. They work by suppressing the activity of the immune system in the skin.
- Topical immunomodulators: Topical immunomodulators are medications that are applied