inflammatory myositis; Inflammatory myopathies are a group of diseases

in which inflammation occurs in muscles and often in organs and tissues other than
muscle. This group includes various diseases whose names contain the term
"myositis," which simply means muscle inflammation:
●Dermatomyositis
●Polymyositis
●Juvenile dermatomyositis
●Juvenile polymyositis
●Myositis that occurs with other systemic (body-wide) rheumatic diseases, such
as mixed connective tissue disease, lupus, and scleroderma
●Autoimmune necrotizing myopathy

The cause of the idiopathic inflammatory myopathies remains undetermined. All are
thought to be due to immune system abnormalities leading to the development of
inflammation in muscle and other tissues.

These are rare disorders, together affecting only about 1 in 100,000 people per year.
More women than men are affected. Although the peak age of onset is in the 50s,
the disorders can occur at any age. The word "juvenile" is used in the name when a
child is affected by myositis.

Clinical manifestations — The main symptom that is common to the inflammatory

myopathies is muscle weakness. Other symptoms that indicate involvement of body
systems other than muscle can occur.

Muscle weakness — Typically patients develop painless weakness of the proximal

muscles—the large muscle groups of the upper arms, thighs, neck, and trunk—in a
symmetric pattern affecting both sides of the body. The smaller distal muscles of the
hands, wrists, feet, and ankles are usually not affected. Patients may notice difficulty
rising from a chair, climbing stairs, or performing tasks overhead such as reaching
up to a high shelf, while grip strength remains normal. At times there may be mild
muscle soreness. The weakness usually develops over several weeks to months.

The throat and upper esophagus consist of muscle identical to the muscles that
control voluntary movement. As a result of weakness in these muscles, some
patients will develop difficulty swallowing or may aspirate food into the lungs, which
can lead to pneumonia.

Skin changes — People with dermatomyositis often develop a rash or other

changes in the skin. Sometimes the rash develops before muscle problems occur. In
some cases, the rash of dermatomyositis appears, but muscle weakness never
develops. Several types of rash may occur:

●Gottron's sign or Gottron's papules – Gottron's sign is a flat red rash over the
back of the fingers, elbows or knees. Gottron's papules are red, often scaly,
bumps overlying the knuckles of the fingers (picture 1).
●Shawl sign – The shawl sign is a widespread, flat, reddened area that appears
on the upper back, shoulders, and back of the neck. It can worsen with
exposure to ultraviolet light.
 ●V sign – The V sign has an appearance similar to that of the shawl sign, but
appears on the front of the chest in the area of skin exposed by a V-necked
sweater.
●Heliotrope rash – The heliotrope rash is located on the upper eyelids and is
often accompanied by eyelid swelling (picture 2). The heliotrope sign is named
for the heliotrope flower, which is violet-colored.
●Nail abnormalities – The nailfolds (the skin around the fingernails) may become
reddened (picture 3) and may develop changes in the blood vessels (picture 4).
●Mechanic's hands – People with dermatomyositis or polymyositis may develop
"mechanic's hands," a roughening and cracking of the skin of the tips and sides
of the fingers, resulting in irregular, dirty-appearing lines that resemble those of
a manual laborer (picture 5).
●Scalp – Changes in the scalp resembling psoriasis may occur in people with
dermatomyositis.

Lung disease — Interstitial lung disease (inflammation of lung tissue) occurs most
commonly in patients with antisynthetase antibodies in their blood. These patients
may develop a cough and shortness of breath with exertion that ranges from mild
symptoms to severe, progressive respiratory distress.

Antisynthetase syndrome — This subgroup of people have one of a family of

antibodies called "antisynthetase" antibodies in their blood. Anti-Jo-1 antibody is the
most common of these antibodies; it is found in about 20 percent of people with
dermatomyositis. The antisynthetase syndrome is characterized by rashes
(particularly mechanic's hands), interstitial lung disease, fever, arthritis, and Raynaud
phenomenon (in which the fingers turn white on cold exposure) in addition to muscle
disease.

Juvenile dermatomyositis — Children with inflammatory myopathy usually but not

always have a dermatomyositis rash. They differ from adults most importantly in that
painful calcium deposits can form on the skin and on the fibrous tissue that wraps
around muscles, called fascia.

Other systemic rheumatic diseases — When myositis accompanies scleroderma

or systemic lupus, the myositis does not always cause symptoms. Some people
have mild muscle weakness, while others have only abnormal blood muscle
enzymes to indicate the presence of the disease.

Cancer — People with inflammatory myopathies, especially adults with

dermatomyositis who do not have antisynthetase antibodies (see 'Antisynthetase
syndrome' above), have an increased risk of cancer. This risk increases with age
and is associated with the same common cancers that affect the general population,
including cancer of the lung, breast, prostate (in men), and ovaries (in women).
Some patients may have tested positive for certain specific antibodies (proteins in
the blood) that are associated with an increased risk of cancer. In most cases,
people newly diagnosed with polymyositis or dermatomyositis should be screened
for cancer. This may include blood tests, chest X-ray, colonoscopy, Pap test,
transvaginal ultrasound, and mammogram (depending on the person's age and sex).

Diagnosis — The diagnosis of the inflammatory myopathies involves a careful

history, a thorough physical exam, and some blood tests. People who have these
conditions often have muscle weakness and/or typical skin changes, and blood tests
showing evidence of muscle damage or the presence of antibody markers that signal
these diseases. Nearly all patients with myositis will have elevation of creatine
kinase (CK) levels. Many will have antinuclear antibodies (ANA) or one of the
antisynthetase antibodies in their blood. Some myositis patients however will be
"serologically silent," meaning they have no antibody markers.

In patients in whom it is difficult to determine the cause of muscle weakness, other

tests may be useful. A magnetic resonance imaging (MRI) scan of the muscles, for
example, can demonstrate changes suggesting muscle inflammation, while an
electromyogram (EMG) can demonstrate abnormal electrical activity in muscles, also
indicating muscle injury. An EMG is usually performed by a neurologist and involves
inserting small needles into muscles, usually of the arms or legs, and recording the
electrical potentials generated by the muscle. EMG testing can help distinguish
weakness due to muscle disease from weakness due to nerve problems.

A muscle biopsy is a procedure that removes a small piece of muscle through an

incision in the skin. The specimen is prepared with special stains and a pathologist
then examines it for evidence of muscle inflammation and for specific characteristics
of polymyositis or dermatomyositis. Muscle biopsy is the most accurate test to
definitively diagnose dermatomyositis or polymyositis but may not be necessary to
establish the diagnosis in cases with typical presentations and characteristic rash.

Other conditions that can cause muscle weakness and need to be distinguished from
the inflammatory myopathies include:

●Hypothyroidism (usually severe cases).

●Drug-induced myopathies – Drugs that can cause myopathy include statin
drugs, used to treat elevated cholesterol; prednisone, in high and prolonged
doses; and colchicine, used on a daily basis in patients with kidney disease.
●Muscular dystrophies, which have specific patterns of weakness and may be
familial.
●Metabolic myopathies – These are rare conditions that are due to abnormalities
in enzymes involved in the metabolism of carbohydrates or fats.
●Electrolyte abnormalities, such as severe potassium depletion.
●Infections (most commonly viral).
●Inclusion body myositis – This is a rare disease that most often affects older
men and causes slowly progressive weakness that tends to involve distal
muscles, such as those in the hands, as well as proximal muscles. The
diagnosis for this condition is established by characteristic changes on muscle
biopsy.

Treatment — Treatment of the inflammatory myopathies is usually initiated with

prednisone. Prednisone is a form of cortisone that in high doses is a very effective
antiinflammatory agent.

In patients treated with prednisone, muscle enzyme levels will begin to fall in a few
weeks and muscle strength usually recovers within a few months. Unfortunately,
high-dose prednisone can also cause numerous side effects, including weight gain
and redistribution of body fat to the face, neck, and abdomen; elevated blood sugar;
mood swings; thinning of the skin; and osteoporosis. For this reason, it is important
to use the lowest dose for the shortest time that controls the disease.

Methotrexate or azathioprine is usually added early in the treatment course in order

to minimize the use of prednisone. While these medications do have some side
effects, they are often effective in controlling the disease long-term and they can be
used safely in the vast majority of patients. Methotrexate is taken orally or by
injection on a weekly basis. Blood counts and liver tests are monitored periodically.
Azathioprine is an oral daily medication and can sometimes cause anemia.

For those patients who are resistant to treatment with either methotrexate or
azathioprine, other treatment options include a combination of both methotrexate
and azathioprine, intravenous immune globulin, mycophenolate, or rituximab.

Hydroxychloroquine (an antimalarial drug related to quinine and chloroquine) is often

effective in the treatment of dermatomyositis rashes.

Since polymyositis and dermatomyositis cannot be cured but can be controlled by

medications, it is important for patients with these diseases to be monitored long-
term for signs of disease reactivation. That way, appropriate adjustments in
treatment can be made. Patients need to have their CK level and muscle strength
tested on a regular basis.

General measures — In addition to medications, people with dermatomyositis or

polymyositis should take precautions to prevent complications related to the disease
and its treatments. These precautions include:

●Osteoporosis prevention – A calcium supplement with vitamin D and a

prescription medication to prevent osteoporosis, such as one of the
bisphosphonates, are often recommended in patients treated with prednisone.
The potential benefits and possible side effects of such medications should be
discussed with a healthcare provider. (See "Patient education: Calcium and
vitamin D for bone health (Beyond the Basics)" and "Patient education:
Osteoporosis prevention and treatment (Beyond the Basics)".)
●Exercise – Physical therapy and rehabilitation should begin soon after the
diagnosis of dermatomyositis or polymyositis to prevent contractures (shortening
of the muscle that can limit joint movement).
●Avoidance of sunlight – Since the rash of dermatomyositis is often worsened by
sun exposure, people with dermatomyositis should protect themselves from the
sun by using sunscreen or seeking shade. (See "Patient education: Sunburn
prevention (Beyond the Basics)".)
●Aspiration prevention – Patients who have trouble swallowing must take care to
avoid inhaling (aspirating) foods and drinks. This may be done by elevating the
head of the bed, by adding thickening agents to drinks, or, in severe cases, by
having a feeding tube placed in the stomach.
●Pneumonia prevention – The medications used to treat dermatomyositis and
polymyositis (glucocorticoids, azathioprine, and methotrexate) weaken the
immune system. This can increase the risk of certain infections, including a type
of pneumonia called Pneumocystis. To decrease this risk, an antibiotic may be
recommended.
Pregnancy and inflammatory myopathy — Little information is available regarding
the impact of pregnancy on inflammatory myopathies or the impact of inflammatory
myopathies on pregnancy. The data that are available indicate that complications of
pregnancy are less likely in women who have inactive disease. Complications may
include a smaller than normal infant, stillbirth, or premature birth.

Women with polymyositis or dermatomyositis who want to become pregnant should

discuss their condition and current medications with a rheumatologist or maternal
fetal medicine specialist before trying to conceive. Some medications, particularly
methotrexate, are not safe for use during pregnancy due to a seriously increased risk
of miscarriage and birth defects.

Dermatomyositis and polymyositis prognosis — The severity of disease in a

person with dermatomyositis or polymyositis is highly variable, ranging from mild
weakness that responds well to treatment to a rapid progression of symptoms that
are unresponsive to all treatments. Less commonly, people with these conditions
improve spontaneously without any treatment.

People with dermatomyositis or polymyositis tend to have a better outcome if they

are treated promptly, have mild muscle weakness, have no difficulty swallowing, and
have no signs of disease in other organ systems such as the heart and lungs.