PMS

د.عقبة Polymyositis

Dermatomyositis
PM & DMS:
It is a diffuse inflammation of C.T affecting the muscle & the skin.
Epidemiology:
* Rare disorder.
* 2-10 / million /year
* All races & ages
* Genetically predispose: HLA-B8 & DR3
Myositis subsets:
1- Adult PM.
2- Adult DMS.
3- Adult PM or DMS associated with malignancy.
4- Childhood DMS.
5-PM associated with other C.T dis.
Adult polymyositis
Clinical features
- ♀/♂--------3/1
- Usually insidious onset & sometime abrupt with rapid progression.
- 3rd – 5th decade.
Presentation:
*Difficult climbing stair or rising from chair
*M.pain & tenderness
*Mild arthralgia & arthritis
*Raynaud s phenomena


Examination of PM:
Clinical exam:
*Weakness of pelvic &/or shoulder girdle m.
*pharyngeal & laryngeal & respiratory m. paralysis ; Dysphagia , Dystonia &
Resp. failure .
*Erythematous rash over elbow & knuckles.
*M.wasting →atrophy – calcenosis - fibrosis→ flexion contracture.
Adult dermatomyositis
Clinical features:
*Common in female.
*Acute or sub-acute m. weakness associated with periorbital oedema & Skin involvement like:
-Heliotropic rash in upper eyelids.
-Photosensitivity .
-Gottron s papules over elbow, knuckles &knee
- Erythematous scaly rash on face , shoulder & Upper arm .
*M.pain & tenderness & wt. loss .
*Arthralgia & mild inflame. Arthritis .

Investigations of PM & DMS :

1.Muscle enzyme estimation is a useful guide of dis. Activity:
-Serum aminotransferase .
- S. Aldolase .
- Creatinine phosphokinase (CPK)
2. Serological examination:
- ANA +ve
- R. Factor +ve
- Anti-jo-1 AB IS presence.
3. Electromyography (EMG).
4. M. Biopsy: fibrosis, necrosis & regeneration with inflam. Cells.
5. MRI to detect active myositis.
6. Screening for malignancy:
- Chest x-ray.
- Mammography.
- U/S .
- GUE.
- S. tumor markers.
Treatment:
Induce remission:
Prednisolone 40-60mg.→ m. enz. fall before clinical improvement.
Maintenance remission:
Prednisolone 10-15mg.
Immunosuppressive drugs if no response.
Supportive parameters:
- Splint
- Physiotherapy to prevent m. contractures.
Prognosis:
Is poor if associated with malignancy & in older patients.


Myositis associated with malignancy
- Less common .
- After age of 40 y.
-Ovarian , Gastric & Nasopharyngeal Ca.
-Insidious onset & become apparent 2-3 years after Ca.
-Clinical features:
similar as PM or DM
- Resection of tumor associated with remission.
Childhood dermatomyositis
Clinical features :
- Age incidence 4-10 y.
- M.weakness & typical rash of DMS.
- M. atrophy – contracture & subcutaneous calcification.
- Recurrent abdominal pain with vasculitis.
Sjogren syndrome
It s an autoimmune disorder of unknown etiology , characterized by lymphatic infiltration of salivary & lacrimal glands leading to Xerostomia & Keratoconjuctivitis sicca.
Rare features:
1- Anemia, Leukopenia, thrombocytopenia.
2- Lymphadenopathy, Hepatomegaly.
3- Hyperglobulinemic purpura, vasculitis, neuropathy.
4- Myositis, fibrosing alveolitis.
5- G.N. & renal tubular necrosis.
Primary S.S:
( Sicca syndrome ) which characterized by:
* Keratoconjuctivitis sicca
* Xerostomia
* Enlarged salivary gland
- Age of onset 40-60 y.
- ♀>♂
- HLA –B8/DR3
Secondary S.S:
It is 2nrdy to auto immune dis. Like:
- R.A
- Chronic active hepatitis
- Myasthenia gravis
- Polymyositis
- Thyroiditis
- SLE
- PSS (Progressive systemic Sclerosis)
- Primary biliary cirrhosis.
Investigations:
1. Salivary flow rate measurement by:
-Parotid sialography.
-Salivary scintigraphy.
2. Schirmer test:
Filter paper wetting in less than 5mm. in 5min. →keratoconj.sicca.
3. Biopsy:
-Lip biopsy from minor salivary gland.
-Biopsy of major salivary gland
4. auto antibodies:
- R.F
- ANA
- SS-A (anti-Ro)
- SS-B (anti-La )
- Salivary gland AB
- Gastric parietal cell AB
- Thyroid AB
Management:
- Artificial tear drops; lacrimal substitution with hypermelose
- Soft contact lenses
- Vaginal lubricants
- Systemic C.S & immunosuppressive drugs .
Mixed connective tissue disease
(MCTD)
- It is characterized by overlapping of clinical features suggesting SLE, PSS & PM.
- It associated very high titer of ANA with specificity for ribonuclease-sensitive extractable nuclear Ag (ENA) , identified as nuclear ribonucleoprotein ( nRNA).
Clinical feature:
- ♀>♂
- Onset 3rd-4th decade or any age.
- Raynaud s phenomena
- Sausage swelling of fingers
- Scleroderma or dermatomyositis
- Mild inflam. Polyarthritis associated with proximal m. weakness & tenderness
- Oseophageal abnormal motility
- Diffuse interstitial pulmonary fibrosis is not uncommon.
- Cardiac, Renal & CNS are very rare.


Investigations:
- ESR ↑
- M.enzyme↑
- ANA +ve
- nRNA +ve -----high titer
Treatment:
Good response to small dose of steroid.

Ideal Group

A.H.Yahia
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