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					PIN GUIDELINE No: 5.01                                                          Page 1 of 4




                       UK PRIMARY IMMUNODEFICIENCY NETWORK
       This document represents the consensus of the PIN Guideline Writing Group.
                 Local variations to these Guidelines may have been made.
 These Guidelines are for the diagnosis and management of the condition stated, for use by
                      Immunologists and Immunology Specialist Nurses.
            Clinical judgement supersedes the Guidelines wherever necessary.




Title                                     Monitoring PID patients on immunoglobulin


Document No                               5


Version No                                1


Date original posted


Date approved by PIN Writing Group


Date approved by PIN Steering Group


Date for review


Withdrawal date                           June 2009


Replaces version No                       This is first version


Filename                                  5.01 Monitoring Ig therapy


Status                                    Draft, in consultation



                   Check this document is the latest version in use!

              A list of current Guidelines is available at www.ukpin.org.uk




                        MONITORING PID PATIENTS ON IMMUNOGLOBULIN
PIN GUIDELINE No: 5.01                                                                  Page 2 of 4


AIM

   To explain how to monitor primary immunodeficiency patients receiving immunoglobulin.

INTRODUCTION

There are a number of key elements of care common to all immune deficient patients,
irrespective of the underlying diagnosis. These areas are:

1. Monitoring for signs of organ damage secondary to the disease process
2. Monitoring the efficacy of treatment
3. Monitoring for risks associated with any blood products given

For management of specific immunodeficiency syndromes, reference should be made to the
relevant Guideline.

WHEN TO USE THIS PROTOCOL

   After the initial visit patients should be seen in the medical outpatients at regular intervals.
   Attendances every 6 months or more frequently as dictated by clinical need are
    suggested.
   Nursing review is undertaken at each visit to outpatients and at any intervening
    attendance for therapy (eg. IVIG).
   The clinician initiating immunoglobulin therapy is responsible for ensuring that monitoring
    takes place, no matter where immunoglobulin is given (for example, as home therapy or
    in another hospital).
   Local PID centres should draw up a flow chart detailing frequencies of monitoring tests
    and recording when this has taken place. These flow charts should be reviewed as part of
    individual risk assessment (see PIN Guideline on risk assessment) and the entire patient
    cohort may be audited.

HOW TO USE THIS PROTOCOL
Monitoring for signs of organ damage secondary to the disease process.
The main area sustaining damage secondary to antibody deficiency is the respiratory tract. In
addition, granulomatous disease can affect the lung, liver, spleen and gastro-intestinal tract.
Evidence for damage to these organs can be monitored in four ways: clinically, radiologically,
physiologically and microbiologically.

Clinical monitoring
 This would include general assessment of well-being, in addition to assessment of the
    impact of organ damage on the patient's quality of life.

Radiological monitoring:
 Chest radiographs are useful for the identification of acute infection or of gross structural
   abnormalities. There is evidence from recent studies that chest radiographs lack the
   necessary sensitivity to identify ongoing lung damage, in particular the presence and
   progression of bronchiectasis [1]. (Evidence Level C)
 High Resolution CT Scans have been demonstrated to be effective in identifying early
   structural changes in the lungs, particularly fibrosis and bronchiectasis, in addition to
   granulomatous disease [1]. The need for CT scanning in asymptomatic patients and the
   frequency of repeat investigation have not been determined. Caution must be exercised
   if radiosensitivity is suspected (ataxia telangiectasia, Nijmegen breakage syndrome,
   adults with T cell disorders and in vitro radiosensitivity). It is anticipated that centres will
   develop local protocols in conjunction with respiratory physicians, to address the above
   issues.

Pulmonary Function Tests: (Evidence Level D)

   The available evidence suggests that there may be a poor correlation between


                         MONITORING PID PATIENTS ON IMMUNOGLOBULIN
PIN GUIDELINE No: 5.01                                                                Page 3 of 4


    abnormalities seen radiologically and the results of PFTs [1-3].
   PFTs in adults are safe and extremely useful for the identification of both reversible and
    irreversible pulmonary disease occurring in primary antibody deficiency.
   PFTs can also be used to evaluate granulomatous disease affecting the lungs.

   PFTs may be more difficult to obtain in children and are not generally possible in younger
    children.
   It is recommended that centres establish local protocols in conjunction with respiratory
    physicians for the performance of PFTs.

Microbiological evaluation (Evidence level D)
 Collection of sputum or nasal secretions is useful in the acute setting. In the paediatric
    setting cough swabs are a more practical way of obtaining respiratory tract secretions. In
    the chronic situation, it is not clear from the published evidence that sputum production is
    associated with worsening pulmonary state [4]. The isolation of pathogenic bacteria,
    viruses or fungi should however prompt treatment in the presence of other clinical
    indicators of acute infection.
 There is no clear published evidence that attempted clearance of pathogenic organisms
    from the respiratory tract of patients with antibody deficiency and established structural
    lung damage is beneficial. Similarly, in the absence of clinical manifestations of infection,
    there is no evidence to indicate the significance, if any, of Pseudomonas spp. colonisation
    in primary antibody deficiency.

Monitoring the efficacy of immunoglobulin treatment:

   There is clear evidence from trials that replacement treatment with immunoglobulin is
    beneficial in primary immunodeficiency (Evidence level B). Significant acute bacterial
    pulmonary infections were significantly prevented in XLA patients treated with IVIG
    sufficient to maintain trough IgG levels above 5g/l [5]. There is similar evidence in CVID
    [6] and some unpublished evidence that increasing the dose of IVIG ((600mg/kg) or
    maintaining a higher trough level (8g/l) may be of additional benefit (evidence level D).
   Paediatricians use a target trough level set at the middle of the normal range for age
    (Evidence level D, expert opinion only).
   Monitoring of trough IgG levels should be performed before infusions of IVIG and action
    taken if levels fall below a defined level. This level may vary between patients, and
    should be adjusted to optimize patient well-being
   There is no evidence to indicate the optimal frequency of monitoring although changes in
    dose may take up to 3 months to stabilise.

Monitoring for risks associated with any blood products given:

   There is a risk of the transmission of blood borne infection, in particular Hepatitis C [7-11]
    from immunoglobulin products (Evidence level C). The aim of this monitoring is the early
    detection of viral infection which may improve response to treatment [12] and may help
    prevent infection of other potential recipients (Evidence level C).
   There is evidence that global monitoring and documentation of batch usage can reduce
    the size of blood borne outbreaks, by rapidly suspending contaminated batches. Batch
    numbers should be stored indefinitely.
   Liver function testing has been used to detect outbreaks of hepatitis C. LFTs are readily
    available and may detect other pathologies. The frequency of testing has not been
    determined but should be undertaken several times per year..
   The saving of patient serum to allow tracing of transmitted infection should run in
    association with a screening program for hepatitis C. Stored serum may also be useful for
    retrospective of other pathogens which may emerge. Serum saves should be done
    annually and when ever immunoglobulin products are changed (Evidence level: D expert
    opinion). The consultant in charge of a given patient should always be the person to
    decide when saved serum should be used for further testing.
   In antibody deficiency, serological testing for HCV testing is unreliable. A recent ESID
    audit has shown that monitoring liver function does not detect all HCV infected patients.



                        MONITORING PID PATIENTS ON IMMUNOGLOBULIN
PIN GUIDELINE No: 5.01                                                               Page 4 of 4


      PID patients on immunoglobulin should therefore be routinely screened for HCV infection
      by HCV PCR.
     There is no available data to indicate the frequency with which RT-PCR for HCV should
      be performed, although it should probably be done at least annually.

RISK MANAGEMENT

Documentation allows us to be sure that therapy is being provided in a safe and effective
manner, patients also have a responsibility in this respect and repeated non-attendance at
clinic should raise concerns over the ability of the unit to provide ongoing therapy without
adequate supervision.

REFERENCES

1.       Kainulainen, L., et al., Pulmonary abnormalities in patients with primary
         hypogammaglobulinemia. J Allergy Clin Immunol, 1999. 104(5): p. 1031-6.
2.       Obregon, R.G., et al., Radiologic findings of adult primary immunodeficiency
         disorders. Contribution of CT. Chest, 1994. 106(2): p. 490-5.
3.       Dukes, R.J., E.C. Rosenow, 3rd, and P.E. Hermans, Pulmonary manifestations of
         hypogammaglobulinaemia. Thorax, 1978. 33(5): p. 603-7.
4.       Kainulainen, L., et al., Viruses and bacteria in bronchial samples from patients with
         primary hypogammaglobulinemia. Am J Respir Crit Care Med, 1999. 159(4 Pt 1): p.
         1199-204.
5.       Quartier, P., et al., Early and prolonged intravenous immunoglobulin replacement
         therapy in childhood agammaglobulinemia: a retrospective survey of 31 patients. J
         Pediatr, 1999. 134(5): p. 589-96.
6.       Busse, P.J., S. Razvi, and C. Cunningham-Rundles, Efficacy of intravenous
         immunoglobulin in the prevention of pneumonia in patients with common variable
         immunodeficiency. J Allergy Clin Immunol, 2002. 109(6 Pt 1): p. 1001-4.
7.       Yap, P.L., et al., Hepatitis C virus transmission by intravenous immunoglobulin. J
         Hepatol, 1994. 21(3): p. 455-60.
8.       Williams, P.E., et al., Transmission of non-A, non-B hepatitis by pH4-treated
         intravenous immunoglobulin. Vox Sang, 1989. 57(1): p. 15-8.
9.       Bjorkander, J., et al., Intravenous immunoglobulin prophylaxis causing liver damage
         in 16 of 77 patients with hypogammaglobulinemia or IgG subclass deficiency. Am J
         Med, 1988. 84(1): p. 107-11.
10.      Bjoro, K., et al., Hepatitis C infection in patients with primary hypogammaglobulinemia
         after treatment with contaminated immune globulin. N Engl J Med, 1994. 331(24): p.
         1607-11.
11.      Lever, A.M., et al., Non-A, non-B hepatitis occurring in agammaglobulinaemic
         patients after intravenous immunoglobulin. Lancet, 1984. 2(8411): p. 1062-4.
12.      Chapel, H.M., et al., Five-year follow-up of patients with primary antibody deficiencies
         following an outbreak of acute hepatitis C. Clin Immunol, 2001. 99(3): p. 320-4.




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