Rheumatology

Rheumatology at Care New England

Rheumatology is a subspecialty of medicine that focuses on the evaluation and treatment of patients for autoimmune and inflammatory diseases that primarily affects the musculoskeletal system, and sometimes affecting other organs, like skin, blood vessels, eyes, and nervous system. These diseases are known as systemic Rheumatic diseases.

Rheumatologists see multiple types of arthritis, Vasculitis, and connective tissue diseases, as well as conditions like Rheumatoid Arthritis, Spondyloarthritis, Psoriatic Arthritis, systemic lupus Erythematous, Sjogren’s disease, scleroderma, among others.

Rheumatologist works closely with other specialists, depending on the nature of the Rheumatologic disease, to ensure that the patient receives the appropriate care.

Care New England Medical Group Rheumatology will provide several consultative services and work closely with other specialists within Care New England to provide the most comprehensive, personalized, and seamless approach for patients to help determine their exact cause of pain and discomfort and provide the best care plan possible.

Contact Information:

Care New England Medical Group Rheumatology

Warwick Location
Kent Hospital, Ambulatory Services Pavilion
455 Toll Gate Road
Warwick, RI 02886

Pawtucket Location
111 Brewster Street
Pawtucket, RI 02860
Multi-specialty

Providence Location 
Opening in September 2021
101 Plain Street
Providence, RI 02903

P: (401) 681-2858
F: (401) 921-6943
Office Hours: M-F 9:00 a.m. - 5:00 p.m.

Rheumatic or Autoimmune Conditions

Arthritis
Connective tissue diseases
Vasculitis
Other Diseases
  • Ankylosing spondylitis
  • Behçet’s disease
  • Adult stills disease

new-look-section

 

Autoimmune Diseases

Symptoms

People with autoimmune diseases can develop a wide range of symptoms from fever to joint pain. Even individuals with the same diagnosis can be affected very differently. Symptoms can be limited to one organ or multiple organ systems. Here are examples of some of the symptoms that are seen in rheumatology:

  • Fever
  • Headache
  • Vision changes
  • Hearing loss
  • Fatigue
  • Depression
  • Anxiety
  • Nose bleeds
  • Oral ulcer
  • Dry mouth
  • Dry eyes
  • Pain with chewing
  • Difficulty breathing
  • Chest pain
  • Cough
  • Joint pain
  • Joint swelling
  • Morning stiffness
  • Reflux
  • Diarrhea
  • Stomach pain
  • Back stiffness
  • Reduced motion
  • Muscle weakness
  • Skin rashes
  • Sensitivity to the sun

There may be other symptoms that affect other functions of the body, including anemia (low level of red blood cells), blood clots, weight loss, loss of appetite, variations in kidney or lung function or changes in coloration of extremities, like fingers.

Diagnosis

The diagnosis of an autoimmune disease includes looking at both patient symptoms and blood work.

Early in the disease process, it can be difficult to obtain the correct diagnosis because the symptoms are subtle and nonspecific. Many symptoms in autoimmune diseases can also be seen in other diseases as well. One of the key features that suggest a process is related to an autoimmune disorder is if symptoms are chronic, relapsing, and have been occurring over 6 weeks.

Lab tests look for elevated inflammatory markers in the blood as well as specific antibodies that are present in certain autoimmune diseases. It is important to correlate lab tests with symptoms. There are some people who have positive antibodies related to this disease process but do have any underlying disease.

Treatment

Autoimmune conditions are chronic and require long-term treatment with immunosuppressive medications to keep the disease under control. Sometimes a flare-up can develop and will require short-acting treatment to help control symptoms.

There are many different medications that can be used to treat autoimmune diseases and it can be a challenge to find the right one that works best for each patient.

It is important to follow up regularly with your physician to monitor symptoms and disease activity by both lab work and physical exam.

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Autoimmune Condition FAQs

 

Gout
What is gout?

Gout is a disease caused by the buildup of uric acid in the joints leading to intermittent episodes of joint pain and swelling. Uric acid is a waste product of purines, which is normally dissolved in the blood and excreted by the kidneys. Purines are organic compounds obtained from our diet that are used in DNA and other biochemical processes. There is certain food like red meat, seafood, and alcohol that have a higher purine content, which increases the risk of gout. Elevated uric acid occurs when the intake of purines is high or when the output through the kidneys is low. This can occur in the setting of kidney disease, medication, or as the body ages. When the uric acid level is above 6.8 mg/dL, it is unable to be dissolved in the blood and will start to precipitate as crystals called urate. When urate builds up in the joint it can lead to inflammation and joint pain. Not even a person with elevated uric acid will have gout. When the uric acid level is above 10 mg/dL there is a 30% chance of a gout attack in the next 5 years.

The crystals in the joint cause an inflammatory response leading to joint pain and swelling. The small peripheral joints are most likely to be affected because they are cooler than the rest of the boy and that allows the uric acid crystals to precipitate easier. Over time if it is not treated it can lead to joint destruction.

Symptoms

Gout is characterized by intermittent episodes of joint pain in the setting of elevated uric acid. Men are affected earlier in life. Attacks can start in the 30s-40s. Women do not usually have symptoms of gout until they are in their 60s. The first toe is the most common joint to be affected first. A gout attack is associated with swelling, warmth, and redness of the joint. The joint can be extremely sensitive to touch. Although the first toe is the most common, gout is can affect any joint. It is progressive with a long-standing disease affecting the hands, ankles, and knees. A woman usually will present first with pain in the hands.

Gout attacks will self-resolve early in the course of the disease after about 2 weeks, even if untreated. If there is long-standing elevated uric acid, then gout attacks will become more frequent and can attack more joints. Eventually, there will be the development of tophaceous gout. Tophi are collections of uric acid crystals. They can appear as chalky masses under the skin around the joints. Uric acid can also deposit in other areas of the body leading to soft tissue swelling around the joint (bursitis), nodules, and kidney stones. The normal uric acid level in the blood is less than 6 mg/dL in females and 7 mg /dL in males. During an acute gout attack, the uric acid level can be falsely low and should be checked 2 weeks after an acute attack.

Diagnosis

Gout is diagnosed by extracting fluid from the joint with a needle and examining it under microscopy. Identifying urate crystals under the microscope is the gold standard for diagnosis. It is important to rule out other causes of acute joint pain, like infection in the joint. This is also done by evaluating the fluid under the microscope. X-ray imaging is not helpful in the initial diagnosis of gout, but it can be useful if there is a long history of gout to evaluate the extent of joint damage.

Treatment

Acute gout attack

The mainstay of treatment of an acute gout attack is anti-inflammatory medications.

  • NSAIDS
  • Steroids (Prednisone )
  • Colchicine (Colcrys/Mitigare)

These medications should be started at the onset of an acute attack and continue until the uric acid level is less than 6 mg/dL.

Uric acid lower therapy

There are many different medications that can be uses to help lower the uric acid. One common medication is, Allopurinol. It is started after a gout attack and should be taken with an anti-inflammatory medication until the uric acid level has normalized. If allopurinol is taken alone, it can lead to a gout attack. It is important to closely follow up with your physician to monitor the uric acid level to adjust therapy.

Low Purine Diet

There are foods that can be eaten and food to avoid in order to help prevent attacks of gout.

Foods to eat include:

  • Eggs
  • Fruit
  • Low fat dairy products
  • Nuts
  • Salmon
  • Vegetables
  • Water
  • Whole grains

Foods to avoid:

  • Alcohol
  • High fructose corn syrup
  • Organ meats, like liver and kidneys
  • Red meat
  • Shellfish
  • Sugar
Lupus
What is lupus?

Lupus, or Systemic Lupus Erythematosus, is a complex autoimmune disease that can affect any organ system in the body.

It is caused by the development of autoantibodies by the immune system. Normal antibodies are produced by the immune system to attack invading pathogens like viruses and bacteria to prevent or stop infection. An auto-antibody is a term used when the antibody attacks its own tissue. The exact cause of why this happens is not known. We do know that there are certain genetic and environmental factors that increase the risk of developing autoantibodies which leads to the develop an autoimmune disease. Specific auto-autoantibodies are associated with the diagnosis of lupus. Lupus presents most commonly in early age. Females are more likely to be affected than males.

Symptoms

There are many different ways lupus can present. Some people have sudden and severe onset of symptoms. While other patients will present subtle with nonspecific symptoms, making it difficult to diagnose.

  • Flu-like symptoms - Persistent fever is one of the most common symptoms of lupus and can be overlooked at first as there are many causes to develop a fever. Fatigue, not feeling well, and weight loss are also common symptoms.
  •  Skin - There are many different skin manifestations that can occur in lupus. The butterfly rash is one of the most common. It is swelling and redness over the cheek and nose but spares the area around the nostrils. The skin is also photosensitive -meaning that a rash develops on the skin after exposure to the sun. Plaques or areas of increased skin pigmentation can also be seen. These findings are seen inactive disease. Many women also have hair loss.
  • Joints - Most patients with lupus have involvement of the joints with symptoms of pain in multiple joints. The affected joints are most commonly the small peripheral joints of the hands. The large joint can be involved as well. The presentation is similar to rheumatoid arthritis, and it can be difficult to differentiate between the two based on exam findings. An important difference is seen on the X-ray. In lupus- there is no destruction of the joint.
  • Kidney - Lupus nephritis is a subtype of SLE that causes damage to the kidney in uncontrolled disease. Symptoms of renal involvement include high blood pressure and lower extremity edema. There can also be protein and blood in the urine. These symptoms occur when their damage to the kidney and a decrease in kidney function.
  • Neurologic - Neurologic symptoms can be subtle and can be overlooked at first to be related to lupus. Headache, depression, anxiety, and mild cognitive impairment are important symptoms in lupus that can be missed. Some patients can also develop seizures and nerve involvement.

  • Heart - The most frequent manifestation of lupus-related heart involvement is pericarditis. This is inflammation of the heart lining. This can lead to symptoms of chest pain or shortness or breath. Inflammation from lupus also increases the risk of developing cardiovascular disease.

  • Lungs - Lupus can involve the lining of the lungs leading to chest pain with breathing. The inflammation can promote fluid to build up in the lungs as well. More seriously inflammation involving the lung can cause symptoms of cough, shortness of breath, and low oxygen levels.

  • Blood (hematologic) - Active lupus will lead to low levels of multiple cell lines including the white blood cell count, which includes all the cells that fight infections. This can lead to an increased risk of infection. Patients can also develop anemia- which is a low hemoglobin level. Hemoglobin is what delivers oxygen to different parts of the body. When the hemoglobin level is low, it can lead to symptoms of fatigue, weakness, and shortness of breath. The platelets also make up part of the cells in the blood. Their role is to help make blood clots to stop bleeding.  When they are low it is known as thrombocytopenia. This can lead to an increased risk of bleeding. There can be the development of Antiphospholipid Antibody. When present this can lead to an increased risk of blood clots. This also leads to a higher risk of miscarriage and stillbirths.
Diagnosis

The diagnosis of lupus is made by doing a comprehensive workup starting with a history of symptoms. A complete physical exam is then done looking for skin, joint, and other manifestations of the disease. In addition, a full laboratory work up should be done to evaluate for an organ disfunction. Antibody testing is done to look for auto-antibodies. The ANA stands for antinuclear antibody, this is an auto-antibody that can attack part of the cells where the DNA is located. The ANA is positive in all cases of lupus, but it can be positive is other autoimmune diseases, and in people without lupus. Approximately 15% of women in the general population have a positive ANA. More specific antibody testing for lupus can be done. Anti-double strained DNA antibody is positive in up to 60% of patients with lupus. This is associated with more severe disease and involvement of the kidneys. The anti-smith antibody is positive in 30% of patient with lupus. There are other antibodies that can be checked as well.   In some cases, a tissue biopsy is needed to obtain a diagnosis.

Treatment

The goal of treatment is to suppress the immune system to prevent auto-antibodies from attacking one’s self and causing damage. For mild disease, the main treatment is a drug called hydroxychloroquine. This was originally an anti-malarial treatment but was found to have mild immune-suppressing properties that have improved the symptoms in lupus. For a severe disease with organ involvement, strong immunosuppression is needed immunosuppressive drugs and steroids.

Myositis or Dermatomyositis
What is dermatomyositis?

Dermatomyositis causes muscle inflammation and skin rash, as well as weakness and swelling. It is different from other muscle diseases because it causes inflammatory skin rashes. The disease can occur at any age, but most often in adults between ages 50 to 70, and women are more likely to be diagnosed. It is also sometimes associated with a connective tissue disorder such as lupus or rheumatoid arthritis. Causes of the disease are unknown, but it can be associated with other autoimmune disorders, cancer, and other health concerns.

Symptoms

The swelling and inflammation causes:

  • Rash that is painful or itches
  • Swelling of the upper eyelids
  • Dark spots that are red and purple on joints
  • Stiff joints
  • Raynaud phenomenon
  • Dry or rough skin
  • Swelling around fingernails
  • Calcium deposits that leave hard lumps
  • Muscle fatigue
  • Trouble swallowing
  • Voice changes
  • Weight loss
  • Muscle aches
  • Fever

In certain instances, someone may have difficulty breaking and have coughing or sensitivity to light.

Diagnosis
  • Blood tests
  • Electromyogram
  • MRI
  • Skin biopsy
Treatment

Depending on symptoms, treatment may include:

  • Skin treatment
  • Physical therapy
  • Anti-inflammatory medication – Steroids or corticosteroids
  • Immunoglobulin – Blood administered through an IV
  • Immunosuppressive medication
  • Surgery
Osteoarthritis
What is osteoarthritis?

Arthritis is a condition that causes pain and inflammation in joints. Osteoarthritis is the most common type. It's a long-term, or chronic, degenerative joint disease that affects adults that are mostly middle-age or older. The condition causes cartilage to break down in any joint of the body with the most common being in the hands, hips, knees or spine.

Causes

OA can be cam be categorized as primary or secondary:

  • Primary osteoarthritis – There is no known cause
  • Secondary osteoarthritis – Caused by another injury, infection, disease or physical deformity

In osteoarthritis, cartilage is broken down in the joint and as this occurs, the bone may thicken and form growths, which are called bone spurs. These limit movement and even pieces of bone can float in the joint or cysts can form.

Risk factors
  • Heredity - Some genetic problems may lead to osteoarthritis.
  • Injury - Severe injury to a joint or overuse
  • Weight
Symptoms
  • Grinding in the joint during movement
  • Joint pain 
  • Joint stiffness
  • Less movement in the joint
Diagnosis
  • Physical examination
  • X-rays
Treatment

Treatment may include: 

  • Exercise
  • Heat treatment
  • Injections of lubricant
  • Medicine
  • Physical and occupational therapy
  • Weight maintenance
  • Surgery
Living with osteoarthritis

There is no cure for osteoarthritis, but there are various things that can be done in order to alleviate the pain of the condition, which may include:

  • Assistive accessories or devices, like walkers, canes, and crutches
  • Equipment, including items that help in reaching objects
  • Exercise
  • Medicine
  • Rest
  • Stretching
Polymyalgia Rheumatica
What is polymyalgia rheumatica?

Polymyalgia rheumatic (PMR) is an autoimmune inflammatory disease that leads to joint stiffness and aching in the shoulder and hips. PMR occurs after the age of 50, with a peak incidence between age 70-80. It is 2-3x more common in females. The development of PMR is due to elevated levels of pro-inflammatory cytokines. Cytokines are cells in the immune system that normally work to fight infections. In PMR these cells are found in higher levels around the joints of the shoulders and hips. It leads to inflammation in the tendons and the bursa (fluid around the joint), which causes aching and stiffness of the joint.

Symptoms

Polymyalgia rheumatica is characterized by symmetrical aching and stiffness of the shoulders, neck, and hips. The stiffness most pronounced in the morning after awakening and lasts for a few hours. If untreated the stiffness can last throughout the day. The stiffness can be severe and cause difficulty getting dressed, or combing hair. There is also stiffness after periods of sitting or inactivity. This is known as a gelling phenomenon. The development of stiffness can be sudden, seemingly occurring overnight, or can occur gradually. A key difference from age-related arthritis is that the symptoms of shoulder and hip pain occur on both sides, whereas age-related arthritis is usually one-sided. In addition, stiffness in arthritis usually will last under 1 hour and is worse with activity.

Since PMR is due to inflammation, it can also lead to systemic symptoms including fever, malaise, weight loss, and fatigue. In blood, there will be signs of inflammation. This can be seen by looking at the sedimentation rate and the CRP. These are two lab tests that are markers for inflammation and are elevated in PMR.

Diagnosis

The diagnosis of PMR is based on criteria looking at both symptoms and lab work. It is important to rule out other diseases like rheumatoid arthritis that can present in a similar way.

The diagnostic criteria include:

  • Age over 50
  • Shoulder and/or pelvic aching and stiffness for more than 45 minutes and present for over two weeks.
  • Elevated levels of inflammatory markers in the blood.
  • Rapid improvement with steroids.
Treatment

The treatment of PMR is low-dose steroids (Prednisone). Symptoms of PMR improve immediately with the initiation of treatment with steroids. To treat the inflammation, it requires a long slow taper of steroids over the period of 6 months to 1 year. Nonpharmacologic treatment includes physical therapy and occupational therapy. It is also an important part of the treatment to regain strength and range of motion of joints. It is important to be aware of the side effects of steroids, which include elevated blood sugar, weight gain, increased appetite, weakness of the bones, thinning of skin, hair loss, high blood pressure, and cataracts.

Temporal Arteritis

A small portion of individuals with PMR will develop temporal arteritis. It is also an autoimmune process due to abnormal inflammation against the blood vessels in the head (vasculitis).

Symptoms of temporal arteritis include:

  •  New-onset headache
  • Sensitivity over the temple area
  • Pain with chewing
  • Vision loss or changes

Temporal arteritis is diagnosed by a biopsy of a small section of the temporal artery. This is done by a surgeon. Treatment involves steroids (prednisone). For temporal arteritis, the steeds are started at a higher dose than PMR.

Psoriatic Arthritis
What is psoriatic arthritis?

Psoriatic arthritis is a type of arthritis linked with psoriasis, a chronic skin and nail disease. Psoriasis causes scaly rashes and thick and pitted fingernails. Symptoms of include joint swelling and inflammation and has similar additional symptoms to that of rheumatoid arthritis.

It comes in five forms:

  • Arthritis affecting joints in fingers and toes
  • Arthritis mutilans – Rare arthritis that damages and deforms joints
  • Asymmetrical arthritis in the hands and feet
  • Psoriatic spondylitis - Arthritis of the lower back and spine
  • Symmetrical polyarthritis
Risk factors

There is no known cause, but genetics, environmental factors, and immune conditions may have an impact.

Symptoms
  • Red and scaly rashes
  • Thick, pitted fingernails
  • Painful joints
  • Inflamed or swollen joints in the fingers and toes
  • Deformities in joints
Diagnosis
  • Erythrocyte sedimentation rate test – Observes how red blood cells fall to the bottom of a test tube. Swelling and inflammation cause clumps and heaviness in the blood.
  • Imaging tests – CT scans, MRI, skin biopsies, ultrasound, x-rays
  • Uric acid – High levels in the blood
Treatment
  • Nonsteroidal anti-inflammatory medication
  • Corticosteroids
  • Immunosuppressive medication
  • Vitamins, including Vitamin D and calcium
  • Exercise
  • Biologic medication
  • Heat and cold
  • Occupational therapy
  • Treatment to ease rash symptoms
  • Surgery for damaged joints
  • Splints
Rheumatoid Arthritis
What is rheumatoid arthritis?

 Rheumatoid arthritis (RA) is an inflammatory arthritis caused by the development of antibodies that attack the joints which leads to pain and swelling. It is a chronic, relapsing disease that is more common in females. If untreated, it can lead to joint destruction and deformity. Rheumatoid arthritis is considered a systemic disease, meaning it can cause inflammation to other areas of the body as well. Joints that are commonly affected in RA are the hands (knuckles), wrists, and elbows. You can also develop pain in the feet, ankles, and knees. Rheumatoid arthritis spares the lower and mid-back, but can affect the neck.

Symptoms of rheumatoid arthritis include joint pain and joint swelling affecting both sides of the body. This pain is worse in the morning and is associated with joint stiffness. This can be described as difficulty making a fist or holding a coffee cup in the morning. The pain improves with activity and throughout the day. Rheumatoid arthritis can cause inflammation in other areas of the body including the lungs, eyes, skin, gastrointestinal tract and kidneys; which can lead to other symptoms besides joint pain, including shortness of breath, hoarse voice, eye inflammation, or rashes.

Diagnosis

The diagnostic criteria of rheumatoid arthritis include both symptoms, laboratory tests, and imaging.

Symptoms include:

  • Swelling of multiple small joint
  • Swelling of large joints.
  • Symptoms have been present for more than six weeks

For signs of inflammation in the blood, there are two tests that are commonly used:

  • Sedimentation Rate (ESR)
  • C- Reactive Protein (CRP)

These are nonspecific markers of inflammation and they can be elevated in the setting of any illness. These lab tests can also be used to evaluate for disease activity when undergoing treatment. Other laboratory tests look at antibody production. This includes rheumatoid factor or CCP antibody.

In correlation with symptoms, these antibodies can point to a diagnosis of rheumatoid arthritis. Early in the disease, sometimes the lab tests will not be positive. Approximately 10% to 20% of people diagnosed with rheumatoid arthritis have negative antibody tests. It is also important to look at x-ray imaging of the joints, especially the hands and feet. Rheumatoid arthritis causes destruction of the joint.

Treatment

The goal of treatment is to improve pain and swelling of the joints to prevent joint destruction.

Acute treatment - The acute treatment of rheumatoid arthritis works to treat the active inflammation in the joints. This is done with steroids such as prednisone. This medication works well in the short term to reduce swelling and pain associated with rheumatoid arthritis. It is used at the start of treatment and can be used during flares of the disease.

Long-term steroid use, however, is associated with many side effects including:

  • Infection
  • Weight gain
  • Diabetes
  • Osteoporosis
  • Hair loss
  • High blood pressure
  • Thin skin

Chronic treatment - The focus of long term treatment is to suppress the part of the immune system that is creating antibodies against the joints that leads to inflammation and destruction. This is done by using a class of drugs called DMARDs (disease modifying anti rheumatic drugs). Methotrexate is one of the most common drugs used, but there are other drugs in the DMARD class that can be trialed as well. In severe disease or when Methotrexate is not effective by itself, we add a class of medications call biologic-DMARDs. This is a newer class of injectable drugs that act by targeting very specific areas of the immune system. Examples of biologics include Humira or Enbrel. Non-pharmacologic treatment with physical therapy and occupational therapy are an important part of treatment as well.

Sjögren Syndrome
What is Sjögren syndrome?

Sjögren syndrome is a systemic autoimmune disorder that is characterized by the development of autoantibodies that attack the glands producing moisture. This is a chronic disorder that leads to dry eyes, mouth, skin, and vaginal dryness- collectively known as sicca symptoms. Sicca comes from Latin, meaning dry. In addition to dryness, patients can also experience systemic symptoms including joint pain, fatigue, and muscle aches. Rarely it can affect large organs. It is more common in women and is most prevalent between ages 40-50. Due to the chronic inflammation with activation of the immune system, there is an increased risk of developing lymphoma.

Sjogren syndrome can occur in the setting of other autoimmune diseases like lupus or rheumatoid arthritis. When this occurs, it is considered a secondary disorder. When Sjögren Syndrome occurs by itself, it is considered primary. Sjogren syndrome was named after Swedish ophthalmologist Henrik Sjögren, who was one of the first physicians to correlate a group of women all having a syndrome of eye dryness, oral dryness, and joint pain in the early 1900s.

Symptoms
  • Dry eyes - Sjogren syndrome leads to severe dry eyes due to auto-antibodies attacking the lacrimal glands. These are the glands that produce tears. People with this disorder are unable to wear contact lenses. There is a gritty sensation or foreign body sensation in the eyes.
  • Dry Mouth - The antibodies attack the parotid gland in the mouth and can lead to swelling of the glands in the cheeks. The dryness in the month is severe and best characterized by being unable to eat a cracker without water. This increases the risk of developing cavities.
  • Dry Skin - Dry skin is due to the attack of the oil and sweat glands of the skin. This can lead to irritation and chronic dry skin. Many different types of rashes can also develop due to Sjögren syndrome.
  • Vaginal dryness - The vagina is another area of the body that can be targeted in Sjögren syndrome. This can lead to painful intercourse.
Extra-glandular symptoms

Sjogren syndrome is a systemic disorder and can affect other organ systems besides the glands. Most commonly patients may have symptoms of joint pain and muscle aches that are related to the inflammation due to the auto-antibodies produced in Sjögren Syndrome. The joint pain can be similar to rheumatoid arthritis- leading to pain and swelling of the small joints of the hands. Although joint pain due to Sjögren’s syndrome is not destructive arthritis.

Diagnosis

The first step in diagnosis is quantifying the severity of the dryness through history and physical exam. There are tests that can be done in the office to evaluate for dryness. An eye doctor should formally evaluate the eyes for any sign of corneal damage under a special light. Dry mouth can be assessed by examination and history of multiple dental cavities.

The next step in diagnosis is looking at lab work for evaluation of auto-antibody production against the glandular tissue. Similar to other autoimmune diseases, it is important to correlate positive auto-antibodies with symptoms. People can have positive antibodies without have the disease. The auto-antibody that is positive in Sjögren syndrome is Anti-Ro/SSA autoantibody. This auto-antibody is part of the diagnosis criteria for Sjögren syndrome. The ANA is another auto-antibody that can be positive, but this is nonspecific and also is positive in other autoimmune diseases as well.

If an initial laboratory test is unrevealing, a biopsy of the salivary gland in the lip can be done to evaluate for inflammation and destruction from the autoimmune process to obtain a diagnosis.

Treatment

Treatment of Sjögren syndrome is aimed at treating symptoms of dryness and preventing further destruction of the glands. Treatment of dry eyes includes using artificial tear drops and cyclosporine drops which help decrease the inflammation. Other strategies to help with symptoms of dry eyes include having the tear ducts plugged to promote tear retention, or wearing glasses with side panels. For treatment of the dry mouth, close follow-up with the dentist for dental cleanings is important to prevent cavities. Sugar-free lozenges can stimulate salvia flow along with having small sips of water to improve the sensation of dryness. If symptoms are not improved with lozenges, then oral medications that stimulate salivary glands can be tried. Vaginal dryness can be treated with topical lubricants and estrogen.

If there is extra glandular involvement of the joints and muscles, then further immunosuppression may be required using oral immunosuppressive agents. Plaquenil and Methotrexate are two drugs that are used in Lupus and rheumatoid arthritis respectively that can be tried in cases of systemic symptoms.

Vasculitis
What is vasculitis?

Vasculitis is a rare autoimmune condition where there is inflammation in the blood vessels due to improper activation of the immune system. The normal immune system functions to create antibodies to attack foreign invaders like bacteria or viruses that would cause illness. In vasculitis, the immune system produces autoantibodies, which attack a cell that is part of the immune defense call the neutrophil leading to hyper-activation. This leads to damage and inflammation of the blood vessels. Since there are blood vessels throughout the body, it can lead to serious organ dysfunction and life-threatening illness if untreated. there are many types of vasculitis, one type is ANCA vasculitis. The type of auto-antibody that is produced is called ANCA (anti-neutrophilic cytoplasmic antibody).

There are two different types of ANCAs.

  • p-ANCA
  • c-ANCA

They correlate with specific types of vasculitis.

ANCA Vasculitis can be broken down in to 3 main types:

  • Granulomatosis with Polyangiitis (GPA) - GPA was formally known as Wegener’s granulomatosis. This disease is characterized by a positive c-ANCA and symptoms most commonly affecting the lungs, kidney, skin, and nerves.
  • Microscopic polyangiitis (MPA)- MPA is very similar to GPA, but the p-ANCA is usually positive in the setting of this illness.
  • Eosinophilic Granulomatosis with Polyangiitis (eGPA)- This type of vasculitis is associated with a history of asthma and a high eosinophil level in the blood.
Symptoms

The presentation of vasculitis can be insidious over months or rapidly progress. It can be difficult to diagnosis initially due to the varying presentations of the disease. Vasculitis can be limited, where only it only affects skin and upper respiratory system. It can also cause systemic disease, damaging major organs like the lung and kidneys.

General symptoms include:

  • Fatigue
  • Weight loss
  • Flu-like-symptoms

Blood vessels of the upper airways and lungs can both be affected. Symptoms include:

  • Chronic sinus infections
  • Nose bleeds
  • Nasal deformity
  • Cough in inflammation of the lungs
  • Shortness of breath
  • Coughing up blood in severe cases

eGPA-patients might frequent asthma exacerbations that are uncontrolled by inhalers.

Vasculitis involving the skin can lead to:

  • Purple rash
  • Ulcerations

When there is the involvement of the kidney it can cause a decrease in kidney function. The inflammation will lead to blood and protein in the urine. This can be seen in a urine study. There can be many different presentations of neurologic symptoms in vasculitis. One nerve can be affected leading to symptoms of pain, weakness or numbness in the distribution of that nerve. Symptoms can also involve multiple nerves. Less frequently seen is inflammation of the tissue of the heart. This can lead to symptoms of chest pain. and shortness of breath.

Diagnosis

Vasculitis is diagnosed through:

  • Clinical symptoms
  • Laboratory findings
  • Tissue biopsy

Due to the varied presentations that can be seen in vasculitis, the diagnosis can be difficult to make. In the setting of a positive ANCA test with classical symptoms suggestive of underlying inflammation on exam and laboratory tests, a presumed diagnosis of vasculitis can be made. It is important that infection is ruled out first. To differentiate between GPA and MPA a tissue biopsy is needed for diagnosis. In GPA, there is granuloma formation at the cellular level. Differentiating between the two types of diseases is important for disease prognosis and long-term treatment.

Depending on which organs have inflammation, will determine where a biopsy is done. A kidney biopsy is usually done if there are signs of vasculitis affecting the kidney. If there is skin or muscle involvement, then a muscle or skin biopsy can be done. A lung biopsy can be done, but it is more invasive.

Treatment

The treatment of vasculitis has advanced significantly over the last 50 years after the introduction of immunosuppressive medication. The goal of initial treatment is to control the active inflammation that is causing organ damage. This is done with high-dose IV steroids. Steroid treatment is continued for at least six months and slowly tapered off while immunosuppressive medications start working.

ANCAs are produced by B-cells, which are a type of white blood cell that is part of the immune system defense. Immunosuppression in vasculitis works by killing immune cells that will produce the ANCAs. Advancements in therapy have led to the development of multiple medications that can directly target the specific immune cells that producing the auto-antibodies. In the case of ANCA vasculitis, the drug Rituximab has been found to be effective in inducing remission by removing the specific b-cell that would lead to disease. There are also other drugs that can be used as well.

There is a risk of relapse is vasculitis if treatment is stopped. The risk is increased in the first five years after the initial diagnosis. Research is still being done on understanding the duration of therapy.

Meet the Team

May Al-Daabil, MD
Janek Karczewski, MD
Isha Shah, MD