Idiopatiske inflammatoriske myopatier - Amboss

Question 1

Hva er idiopatiske inflammatoriske myopatier (IMM)?

Answer 1

Question 2

Hva er definisjonen av dermatomyositt?

Answer 2

Question 3

Hva er definisjonen til overlapsmyositt (“Overlap myositits - OM”)?

Answer 3

Question 4

Hva er definisjonen til immunmediert nekrotiserende myopati (“Immune-mediated necrotizing myopathy - IMNM”)?

Answer 4

Question 5

Hva er definisjonen til inklusjonslegeme myositt (Inclusion body myositis - IBM”)?

Answer 5

Question 6

Hva er definisjonen til polymyositt?

Answer 6

Question 7

Hva er definisjonen til juvenile idiopatisk inflammatorisk myopati (“Juvenile idiopathic inflammatory myopathy (JIIM)?

Answer 7

The most common JIIM is juvenile DM.

Question 8

Hvordan er epidemiologien til IIM?

Answer 8

Question 9

Hvilken etiologi har idiopatiske myopatier?

Answer 9

Question 10

Hvilken krefttype er mest assosiert med dermatomyositt?

Answer 10

Ovarian cancer is reported as one of the most commonly associated malignancies.

Question 11

Er klinikken lik ved alle IIM?
Hvilke kategorier deler man symptomene inn i?

Answer 11

Kliniske manifestasjoner av IIM varierer veldig mellom subtypene, og innenfor hver sykdom.

Symptomene kan grupperes inn i tre kategorier:

Muskelsvakhet

Hudaffeksjoner

Systemiske manifestasjoner

Symtpomtrykk:

Pas. trenger ikke å oppleve symptomer fra alle kategoriene, og alvorligheten av symptomene varierer fra mild til alvorlig.

Question 12

Hvilken muskelaffeksjon kan IIM gi?

Answer 12

Up to 30% of patients with dermatomyositis report myalgia. Weakness in the oropharyngeal muscle is present in up to 50% of patients with dermatomyositis and is very common in inclusion body myositis, where it may be the presenting symptom.

Question 13

Hvilke hudsymptomert kan IIM gi?

Answer 13

Periungal telangektasier; Dilated capillaries around the fingernails.

Question 14

Hvordan er erytemet ofte beskrevet ved IIM?

Answer 14

The erythema is often described as violaceous (blue-purple) in color, but may appear as more subtle hyperpigmentation on darker skin colors.

Question 15

Hva skiller hudsymptomene i ansiktet ved IIM fra SLE?

Answer 15

Cutaneus features may mimic malar rash but, in contrast to SLE, it affects the nasolabial folds.

Question 16

Hva viser bildet?

Answer 16

Question 17

Hva viser bildene?

Answer 17

Question 18

Hva viser bildet?

Answer 18

Question 19

Hva viser bildet?

Answer 19

Question 20

Hva viser bildet?

Answer 20

Question 21

Hva viser bildet?

Answer 21

Question 22

Hvilke systemiske manifestasjoner kan IIM gi?

Answer 22

GI symptoms are due to intestinal vasculopathy.

Question 23

Hva er karakteristisk ved det kliniske bildet ved dermatomyositt og polymyositt?

Answer 23

In DM, up to 20% of cases, muscle features are absent.

Question 24

Hvordan er klinikken ved hhv.:
- Antisynthetase syndrom
- IMNM
- IBM
- JIIM

Answer 24

With antisynthetase syndrome, clinical features vary depending on the subtype involved. Other cutaneous symptoms can also be present.

Question 25

Hvilke ord bør man huske på hos pas. med IIM og deres ADL?

Answer 25

Question 26

Gi et oversiktbilde av dermatomyositt og polymyositt

Answer 26

Question 27

Hva er de generelle prinsippene for å diagnostisre IIM?

Answer 27

Multiple sets of diagnostic criteria for IIM have been proposed over the years, all with various limitations. PM is reported to be overdiagnosed; many patients with this diagnosis are likely to have another type of IIM.

Question 28

Hvilke begrensninger har “The Peter and Bohan” kriteriene?

Answer 28

Limitations include no established definitions of skin findings, the inclusion of nonspecific criteria such as EMG findings, and, because they were developed prior to the development of myositis-specific antibodies, an inability to diagnose other forms, e.g., inclusion body myositis.

Question 29

Hvilke rutinelab. prøver bør tas ved utredning av IIM?

Answer 29

Elevations in CK are typically ≤ 50-fold in DM and PM, > 10-fold and ≤ 100-fold in IMNM, and < 10-fold in IBM. Elevation in liver chemistry are a result of muscle breakdown, not liver damage.

Question 30

Hvilke antistofftester er aktuelle ved utredningen av IIM?

Answer 30

ANAs; These include anti-Ro/SSA, anti-La/SSB, anti-Smith, anti-RNP, anti-Scl-70, and anti-centromere antibodies. MSAs are specific but not very sensitive for IIM. These tests are generally performed in reference laboratories and results may take several weeks to come back.

Question 31

Gi en oversikt over hvilke antistofftester som er anbefalt ved de ulike IIM?

Answer 31

Previously, anti-SRP was associated with PM. However, this association has now been disproved.

Question 32

Hvilke andre prosedyrer er aktuelle ved utredningen for IIM?

Answer 32

Muskelbiopsi

If muscle biopsy is not possible or yields equivocal findings in DM, skin biopsy can be performed. Findings may resemble those in SLE or dermatitis.

Elektromyografi

Irritabelt myopatisk mønster ved alle IIM.

Question 33

Hvordan utfører man en muskelbiopsi ved IMM, og hva ser man etter?

Answer 33

If the muscle is wasted, biopsy will show end-stage muscle, from which it is impossible to determine an underlying cause. A proposed mechanism is that antibodies directed at endothelial cells cause capillary inflammation and necrosis, eventually leading to muscle hypoperfusion and atrophy.

Question 34

Hva ser man etter ved EMG ved utredningen av IIM?

Answer 34

Question 35

Sett på navn

Answer 35

Question 36

Hva viser bildet?

Answer 36

Question 37

Hva viser bildet?

Answer 37

Question 38

Hva viser bildet?

Answer 38

Question 39

Hvilke andre organmanifestasjoner bør utredes ved mistanke om IIM?

Answer 39

Malignancies are found in up to 25% of patients with DM and 10–15% of patients with PM; risk is highest within the first year of IIM diagnosis (especially for DM) and diminishes over time.

Question 40

Hvilke myopatier har er diff.diagnose ved IIM?

0

Answer 40

DM malignancies; E.g., adenocarcinomas of the cervix, ovaries, lung, and stomach.

Question 41

Hvilke andre tilstander er diff.diagnose til myopati?

1

Answer 41

Polymyalgia rheumatica (PR); Peak incidence: > 70 years (rare in patients < 50). ESR specifically > 50 mm/h. With hypothyroid myopathy the sign of hypothyroidism is e.g., fatigue, bradykinesia, constipation, weight gain, cold intolerance.

Question 42

Hvilke medikament-induserte myopatier kan være en diff.diagnose ved IIM?

2

Answer 42

Adverse effets of glucocorticoid therapy is e.g., weight gain, glaucoma, high blood pressure, mood changes. The muscle biopsy in Alcohol-induced myopathy is will most likely show atrophy of type II fibers. The biopsy in Cocain-induced myopathy would show intramuscular vacuolar changes.

Question 43

Hvilke andre diagnoser kan være en diff.diagnose til IIM?

3

Answer 43

Question 44

Hvordan går man fram med behandling ved all IIM?

Answer 44

Supportive therapy helps patients to avoid muscle contracture and maintain muscle function and overall mobility. Treatment is supportive only for patients with IBM.

Question 45

Hvordan er den farmakologiske behandlingen ved IIM?

Answer 45

Controlled trials for these therapies are lacking, and no treatment guidelines exist. Treatment regimens are based on expert opinion and clinical judgment. Pharmacological therapy should be combined with tailored physical therapy programs. For mild cases, some specialists may choose to start steroids as monotherapy and reserve a steroid-sparing immunosuppresive agent as second-line medications.

Question 46

Hvilke komplikasjoner kan oppstå pga. IIM?

Idiopatiske inflammatoriske myopatier

Answer 46

Due to respiratory muscle weakness; interstitial lung disease is an important complication, which arises from respiratory failure that causes pneumonia. Myocarditis causes arrhythmia and pathologic conduction. Weakness of the striated musculature in the upper third of the esophagus that often occurs in older patients and may lead to aspiration pneumonia.