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Comparison 1 Ciclosporin versus placebo at six months, Outcome 1 Six months.
Figuras y tablas -
Analysis 1.1

Comparison 1 Ciclosporin versus placebo at six months, Outcome 1 Six months.

Comparison 2 Ciclosporin versus placebo ‐ change in QMG at six months, Outcome 1 Six months.
Figuras y tablas -
Analysis 2.1

Comparison 2 Ciclosporin versus placebo ‐ change in QMG at six months, Outcome 1 Six months.

Table 1. Summary of Quantitative MG Score for Disease Severity (QMG)

Items tested

Time to develop diplopia with prolonged lateral gaze

Time to develop ptosis with prolonged upgaze

Facial strength

Ability to swallow 4 oz (1/2 cup) of water

Speech after counting aloud from 1 to 50

Time patient can outstretch either arm

Vital capacity

Right and left grip strength

Time patient can lift head 45° in a supine position

Time patient can outstretch either leg

Figuras y tablas -
Table 1. Summary of Quantitative MG Score for Disease Severity (QMG)
Table 2. Summary of MGFA Postintervention Status Scale

Category

Clinical state

Complete Stable Remission (CSR)

No symptoms or signs of MG. Not received therapy for MG for at least one year.

Pharmacologic Remission (PR)

Same as above, but continues to take some form of therapy for MG. Patients on cholinesterase inhibitors are excluded as their use suggests the presence of weakness.

Minimal Manifestations (MM)

No symptoms of functional limitations, but has some weakness on examination.

Minimal Manifestations MM‐0

Not received MG treatment for at least one year.

Minimal Manifestations MM‐1

Continues to receive some form of immunosuppression, but no cholinesterase inhibitors or other symptomatic therapy.

Minimal Manifestations MM‐2

Received only low‐dose cholinesterase inhibitors (<120 mg pyridostigmine/day) for at least one year.

Minimal Manifestations MM‐3

Received cholinesterase inhibitors or other symptomatic therapy and some form of immunosuppression during the past year.

Change in status

Improved (I)

A substantial decrease in pretreatment clinical manifestations, or a sustained substantial reduction in medications, or a specific decrease in QMG score, as defined in the protocol.

Unchanged (U)

No substantial change in pretreatment clinical manifestations or reduction in medications, or a change in QMG score, as defined in the protocol.

Worse (W)

A substantial increase in pretreatment clinical manifestations, or a substantial increase in medications, or a specific increase in QMG score, as defined in the protocol.

Exacerbation (E)

Fulfilled criteria of CSR, PR or MM, but subsequently developed clinical findings greater than permitted by these criteria.

Died of MG (D of MG)

Died of MG, of complications of therapy, or within
30 days of a thymectomy.

Figuras y tablas -
Table 2. Summary of MGFA Postintervention Status Scale
Table 3. MGFA Clinical Classification

Class

Definition

Class I

Any ocular muscle weakness
May have weakness of eye closure
All other muscle strength is normal

Class II

Mild weakness affecting other than ocular muscles
May also have ocular muscle weakness of any severity

Class IIa

Predominantly affecting limb, axial muscles, or both
May also have lesser involvement of oropharyngeal muscles

Class IIb

Predominantly affecting oropharyngeal, respiratory muscles, or both
May also have lesser or equal involvement of limb, axial muscles, or both

Class III

Moderate weakness affecting other than ocular muscles
May also have ocular muscle weakness of any severity

Class IIIa

Predominantly affecting limb, axial muscles, or both
May also have lesser involvement of oropharyngeal muscles

Class IIIb

Predominantly affecting oropharyngeal, respiratory muscles, or both
May also have lesser or equal involvement of limb, axial muscles, or both

Class IV

Severe weakness affecting other than ocular muscles
May also have ocular muscle weakness of any severity

Class IVa

Predominantly affecting limb and/or axial muscles
May also have lesser involvement of oropharyngeal muscles

Class IVb

Predominantly affecting oropharyngeal, respiratory muscles, or both
May also have lesser or equal involvement of limb, axial muscles, or both

Class V

Defined by intubation, with or without mechanical ventilation, except when employed during routine postoperative management. The use of a feeding tube without intubation places the patient in class IVb

Figuras y tablas -
Table 3. MGFA Clinical Classification
Table 4. Methodological Quality Scores

Study ID

Alloc concealment

Patient blinding

Observer blinding

Desc of clin status

Explicit outcomes

Baseline difference

Follow‐up

Statistical power

Bromberg 1997

B

C

C

B

A

C

B

C

De Feo 2002

A

A

A

B

A

A

A

B

Gajdos 1993

A

C

C

A

A

B

A

B

Meriggioli 2003b

A

A

A

A

A

C

B

C

Nagane 2005

C

C

C

A

A

A

A

B

Palace 1998

A

A

A

A

A

A

A

B

Schalke 1990

C

A

A

C

A

A

A

B

Tindall 1987

A

A

A

A

A

A

A

B

Tindall 1993

A

A

A

A

A

A

A

B

Figuras y tablas -
Table 4. Methodological Quality Scores
Comparison 1. Ciclosporin versus placebo at six months

Outcome or subgroup title

No. of studies

No. of participants

Statistical method

Effect size

1 Six months Show forest plot

2

59

Risk Ratio (M‐H, Fixed, 95% CI)

2.44 [1.13, 5.27]

Figuras y tablas -
Comparison 1. Ciclosporin versus placebo at six months
Comparison 2. Ciclosporin versus placebo ‐ change in QMG at six months

Outcome or subgroup title

No. of studies

No. of participants

Statistical method

Effect size

1 Six months Show forest plot

2

59

Mean Difference (IV, Fixed, 95% CI)

‐0.34 [‐0.52, ‐0.17]

Figuras y tablas -
Comparison 2. Ciclosporin versus placebo ‐ change in QMG at six months