IBM, PM, DM, JDM

[Guest guest]

Hello Everyone.... Boy did I have the rain today. I got over an inch

which is really good for us. But the it knocked me off my web twice so

I thought I had better not push it and get off. Since we have people

on this list with different types of myositis, I've copied them down

for you. Have a good evening...

Vicki

Dermatomyositis

Dermatomyositis (DM) is the most easily recognized of the inflammatory

myopathies due to its distinctive rash. This rash occurs as a patchy,

dusky, reddish or lilac rash on the eyelids, cheeks, and bridge of the

nose, and on the back or upper chest, elbows, knees and knuckles. Some

people with dermatomyositis develop calcified nodules or hardened bumps

under the skin. The rash often precedes muscle weakness. Muscle

weakness usually develops over a period of weeks but may develop over

months or even days. The weakness initially affects those muscles

closest to and within the trunk of the body, including neck, hip, trunk,

and shoulder muscles. Difficulty swallowing occurs in at least one third

of DM patients. Whereas less than 25% of adults report muscle pain, more

than 50% of children with DM complain of muscle pain and tenderness.

Dermatomyositis can occur at any age from childhood to adulthood and is

more common in females than males. High dose prednisone (an

immunosuppressant) has been an effective treatment for many patients.

Other nonsteroidal immunosupressants such as azathioprine and

methotrexate are often used. Unfortunately, these drugs have adverse

side effects, especially after prolonged use. For patients who do not

respond well to prednisone, intravenous administration of

immunoglobulins (IVIg) has also proven effective.

 

Polymyositis

Polymyositis (PM) does not have the characteristic rash of

dermatomyositis (DM). Onset of muscle weakness usually progresses slower

than DM. Proximal (nearest to the trunk of the body) limb and neck

muscles are weakened; involvement of distal (farthest from the trunk of

the body) muscles varies. Difficulty in swallowing is common in PM.

Inability to breathe due to muscle failure is uncommon but occurs more

often in PM than in dermatomyositis or inclusion body myositis. As many

as one third of PM patients have muscle pain, but it is rarely the chief

complaint. PM rarely affects people under the age of 20 but cases of

childhood and infant polymyositis have been reported. More women than

men are affected with PM. High doses of prednisone (an

immunosuppressant) have been an effective treatment for many patients.

Other nonsteroidal immunosupressants such as azathioprine and

methotrexate are also prescribed. Unfortunately, these drugs have

adverse side affects, especially after prolonged use. For patients who

do not respond well to prednisone, intravenous administration of

immunoglobulin (IVIg) might be effective.

 

Inclusion Body Myositis

Inclusion body myositis (IBM) is very similar to polymyositis. In fact,

many doctors believe patients diagnosed with PM that do not respond to

treatment actually may have IBM. The only definitive test for IBM is a

muscle biopsy. Onset of muscle weakness in IBM is usually very gradual,

taking place over months or years. It is different from PM in that both

proximal and distal muscles are affected. Typical findings include

weakness of the wrist flexors and finger flexors. Atrophy, or shrinking,

of the forearms is characteristic. In the legs, atrophy of the

quadriceps muscle is common with varying degrees of weakness in other

muscles. Difficulty swallowing occurs in about half the patients

afflicted with IBM. Facial muscle weakness is present in a minority of

patients. Falling is often the first noticeable symptom of IBM.

Symptoms of IBM usually begin after age 50, although no age group is

excluded. IBM occurs more frequently in men than women. About one in ten

cases of IBM may be hereditary. Unfortunately, there is as yet no known

treatment for people with IBM. Intravenous immunoglobulin (IVIg) has

shown some preliminary evidence for a slight beneficial effect in a

small number of cases. New drugs and other avenues are being explored.

Prescribed physical therapy may be helpful to maintain mobility.

 

Juvenile Myositis

Juvenile idiopathic inflammatory myopathy (JIIM) or juvenile myositis

(JM) most often presents itself as dermatomyositis (JDM) with its

typical rashes and muscle weakness. There are fewer cases of juvenile

polymyositis (JPM), inclusion body myositis (JIBM) and other clinical

forms of myositis reported. The JDM rash precedes muscle weakness

greater than 50% of the time. In both cases,(JPM and JDM) muscle

weakness usually develops over a period of months, weeks or days. The

weakness being proximal (closest to and within the trunk of the body)

primarily involves neck, hip, trunk and shoulder muscles, but may also

include distal muscles. Dysphagia (difficulty swallowing), dysphonia

(hoarseness), abdominal pain and arthritis can also occur with this

disorder. Muscle pain is seen in approximately 50% of children with

myositis. Corticosteroids are effective in the majority of patients and

remission with complete withdrawal of medication can be anticipated in a

large percentage of patients. Patients with severe weakness may need

longer periods of treatment. For those patients who experience

dose-limiting side effects of corticosteroids or are unresponsive, there

are other treatments available, including intravenous immune globulin

infusions. It is important to diagnose these patients and start

treatment as soon as possible. The parents and the doctor of a child

with myositis will also want to consider a rehabilitation program with a

team of professional experts in this field.