Immunomodulatory therapy of CIDP https://youtu.be/Z9iCYLOM8WM?si=iXTr7NkfRPuxYNGh

1. Treatment of chronic
inflammatory
polyneuropathy
By
Dr mohammud ibraheem

2. Before starting treatment
An essential aspect of the management of CIDP is the assessment of
patients at baseline and at follow up visits after treatment, using objective
and validated means of determining the severity of the neuropathic
deficits.
Disease heterogeneity and subtypes of CIDP, different time, and the
degree of response to IVIg may require individualized treatment regimen.

3. Chronic Inflammatory Demyelinating
Polyneuropathy (CIDP) Assessment
Tools
1. Inflammatory Neuropathy Cause and Treatment (INCAT)
Disability Score..2001
2. Inflammatory Rasch-built Overall Disability Scale (I-RODS)..
2011
3. Medical Research Council (MRC) Sum Score

4. Lines of treatment
The mainstay of treatment for chronic inflammatory demyelinating
polyradiculoneuropathy (CIDP) is:
a. Immunomodulatory intervention
b. Immunosuppressive intervention

5. Immunoglobulines

6. IVIG
The initial course of IVIG is 2 g/kg over 2–5 days (0.4g/kg/day).
followed by a maintenance dose of 1 g/kg every 3 or 6 weeks.
for elderly patients and patients who have impaired renal or
cardiovascular function or who have high serum viscosity; the schedule
is 0.4 g/kg daily for 5 days. A second dose within 3–4 weeks should be
given, the positive effect may require 6–8 weeks and two to three
courses

7. Subcutaneous
immunoglobulin
SCIG provides more even physiological IgG levels with less “drop
off” period before next dose and less of a sharp peak in IgG levels
after dose. It also has decreased severity and frequency of adverse
side effects.
SCIG is more convenient for patients to administer at home, it
requires self-injection and has potential injection site reactions.

8. Subcutaneous
immunoglobulin
A recent randomized, double-blinded, placebo-controlled trial
confirmed that SCIG administered subcutaneously via infusion
pump at a weekly dose of 0.4 g/kg or 0.2 g/kg is effective as an
alternative maintenance therapy for patients who were responsive
to IVIG treatment

9. Side effect of IVIG
Adverse reactions occur in less than 10% of patients.
1. Headache is the most common infusion related reaction and is mild
to moderate.
2. Severe headache, typical migraine attacks, and nausea are less
common.
3. Myalgias last 1–2 days after infusion.
4. Chills
5. Aseptic meningitis is rare but could be severe.
6. Thrombotic events including stroke, MI, retinal vein occlusion, and
DVT occur in patients with cardiovascular risk factors and
increased serum viscosity, particularly with infusion rates >0.4
g/kg/day.
7. Worsening RF in patients with preexisting renal disease, especially
the elderly, and those with DM and hypovolemia

10. Side effect of IVIG
This complication is associated with IVIG products containing high
concentrations of sucrose.
Precautions before infusion
1. Premedication with diphenhydramine or IV corticosteroids or both
2. Close monitoring of renal function,
3. Correction of hypovolemia,
4. Discontinuation of concomitant nephrotoxic drugs,
5. Use of products without sucrose are measures to prevent renal
tubular necrosis in patients with preexisting kidney disease.
6. The serum IgA level is determined before the 1st infusion because
those with very low IgA levels may have allergic or anaphylactic
reactions during later infusions.

11. FDA approved
immunoglobulines
1. Gamunex
2. Privigen..2017
3. Subcutaneous immune
globulin (SCIg) (Hizentra)
..2018

12. Corticosteroids

13. Corticosteroid
I. Daily single dose therapy
Daily single dose oral prednisone is started at 60–80 mg (1–1.5 mg/kg
for children). Improvement can be anticipated to start within 2 months
but may not be evident till 3–5 months.
Following improvement, the dose may be converted to an alternate day
or continues on single dose schedule with reducing the dose by 10
mg/week.

14. Corticosteroid
II. High dose pulsed therapy
High intermittent IV methylprednisolone (1000mg/day for 3 days
followed by 1000mg/week for 4 weeks) has beneficial effects that are
equal to those of oral prednisone and the SE profile and cost of
treatment are less.
Pulse oral dexamethasone (40 mg/day × 4 days every 4 weeks) resulted
in a similar remission rate and SE as oral prednisone, but the patients
improved twice as fast

15. Corticosteroid
High dose is maintained until a remission or plateau phase is
achieved. More than 50% of patients reach this point by 6 months.
After attaining maximum benefit, a slow taper of prednisone (10
mg/month followed by 5-mg decrements at doses below 50 mg on
alternate days) can then begin.

16. Corticosteroid
Some patients are exquisitely sensitive to reduction in corticosteroid
dosage, in which case this must be reduced slowly to avoid producing a
severe relapse. Patients may need alternate-day prednisone (10–30 mg)
for years to suppress disease activity.
The clinical improvement and side effect profile guides to the rapidity
of the taper.

17. Corticosteroid
SEs of prolonged corticosteroids use are significant.
1. Osteoporosis causing vertebral compression fractures,
2. Obesity,
3. Diabetes, Hypertension,
4. Cataracts are the most common long-term complications
5. Increased intraocular pressure,
6. Aseptic necrosis of bone,
7. Peptic ulcer disease,
8. Susceptibility to infection.

18. Corticosteroid
Precautions taken to diminish complications include
1) A low-sodium (2 g/day)
2) Low carbohydrate diet
3) Proton pump inhibitors
4) Calcium and vitamin D supplements should be initiated within a
few months to limit osteoporosis, and bone density should be
monitored
5) Bisphosphonates or nasal calcitonin is beneficial for patients with
osteopenia or osteoporosis.

19. plasmapheresis

20. Plasmapheresis
Plasma exchange has a benefit for both chronic progressive and
relapsing course.
10 plasma exchanges performed over 4w resulted in substantial but
transient improvement in 80% of patients. Improvement began within
days of starting therapy, yet 70% of responders relapsed within 14
days after plasma exchange is stopped.

21. Plasmapheresis
The optimal schedule for plasma exchanges has not been
established and probably varies from patient to patient.
A common approach employs 3 exchanges (50 mL/kg) weekly for
the first 2 weeks, followed by 1 or 2 exchanges/week from the 3rd
through the 6th week. Then the treatment frequency is adjusted
according to clinical response.

22. Plasmapheresis
Plasmapheresis is difficult to be maintained for months or years, and
the majority of patients needing prolonged plasmapheresis require the
addition of prednisone for lasting benefit and stabilization.
Venous access problems overcome by placement of central venous
catheters, although this approach carries the risk of pneumothorax,
hematoma, brachial plexus injury, and serious infection.

23. Steroids, plasmapharesis or
IVIG
The beneficial effect of IVIG is equivalent to plasmapheresis. Both
treatments are equally efficacious but short lived, and most patients
required continued intermittent treatment for sustained improvement.
One trial compared IVIG (2 g/kg given over 1 or 2 days) with oral
prednisolone (60 mg for 2 weeks followed by a taper over 4 weeks).
Both treatments resulted in improvement after 2 and 6 weeks, although
IVIG tended to be slightly superior to oral prednisolone.

24. Steroids, plasmapharesis or
IVIG
A trial comparing the 6-month of IVIG to intravenous
methylprednisolone showed that IVIG was more effective and better
tolerated than steroids during the first 6 months of treatment.
However, when effective, steroids were more likely to induce
remission and less frequently associated with deterioration after
therapy discontinuation than IVIG

25. Thank you