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Dermatology

Common Variable Immunodeficiency Treatment & Management

Updated: Mar 13, 2018
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more…
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Sections
Common Variable Immunodeficiency
  • Sections
    Common Variable Immunodeficiency
  • Overview
    • Practice Essentials
    • Background
    • Pathophysiology
    • Etiology
    • Epidemiology
    • Prognosis
    • Patient Education
    • Show All
  • Presentation
    • History
    • Physical Examination
    • Complications
    • Show All
  • DDx
  • Workup
    • Laboratory Studies
    • Imaging Studies
    • Other Tests
    • Procedures
    • Histologic Findings
    • Staging
    • Show All
  • Treatment
    • Medical Care
    • Surgical Care
    • Consultations
    • Prevention
    • Long-Term Monitoring
    • Show All
  • Medication
    • Medication Summary
    • Immunoglobulins
    • Show All
  • Questions & Answers
  • References

Treatment

Medical Care

The mainstay of treatment for common variable immunodeficiency (CVID) is Ig replacement therapy. Although expensive, Ig replacement therapy stops the cycle of recurrent infections. See the image below.

Intravenous immunoglobulin infusion. Courtesy of W

Intravenous immunoglobulin infusion. Courtesy of Wikimedia Commons (Steve Brew, own work).

Ig may be administered intravenously or subcutaneously. Solutions of 3-12% intravenous immunoglobulin (IVIG) can be used on a regular basis to maintain a trough level of 400-500 mg/dL in adults. A dose of 400-600 mg/kg every 2-4 weeks is usually required. In patients with structural lung damage, a trough level of 700-800 mg/dL is required.

A solution of 16% subcutaneous injection of IV immunoglobulin (SCIG) is also an effective treatment in patients with poor intravenous access. As expected, the volume required to achieve adequate trough levels is much higher with SCIG than with IVIG. A dose of 160 mg/kg/wk is comparable to an IVIG dose of 400 mg/kg/mo.

Adverse reactions to Ig administration must be monitored during therapy. The most common reactions include backache, nausea, vomiting, chills, low-grade fever, myalgias, and fatigue. Adverse effects occur within 30 minutes of the infusion and usually last for several hours. Slowing the rate of infusion or interrupting the infusion for a few minutes greatly helps in preventing symptoms. The effects can be treated with antipyretics, diphenhydramine, and/or corticosteroids. Although anaphylactic reactions to IVIG are uncommon, patients with IgA deficiency have an increased risk for these effects. Long-term intravenous access is not recommended because it can increase the risk of infection.

The transmission of infectious agents during infusion has caused problems in the past. Although no cases of HIV infection have been linked to Ig therapy, the transmission of hepatitis C virus has been reported. Current methods of viral inactivation help prevent transmission. These methods include treatment with organic solvents and detergents, pasteurization, and storage at a low pH. In the United States, Ig products are derived from pooled human plasma, which undergoes a manufacturing process that includes cold ethanol fractionation and viral inactivation steps.

In most patients, CVID responds well to Ig therapy. The recurrence of infections, arthritic symptoms, and the severity and/or incidence of the autoimmune disease are reduced. Gastrointestinal disease shows little improvement with IVIG. In some patients with severe autoimmune disease, the concurrent use of steroids or other immunosuppressive drugs may be needed.

Cyclosporin A has been successfully used in patients with CVID and lymphoid interstitial pneumonitis. The administration of anti-CD20 monoclonal antibody has been used to treat autoimmune thrombocytopenia and neutropenia. Studies are underway to evaluate the efficacy of IL-2 administration in conjunction with polyethylene glycol. Results of early in vitro studies show an increase in Ig production by B lymphocytes.

Antimicrobial therapy should be initiated at the first sign of infection. A narrow spectrum of drugs should be used when culture and sensitivity results are available. The prophylactic use of antibiotics should be avoided because of an increased risk of infection with fungi or other resistant organisms.

Specific therapy is often necessary to target the organ system involved. For instance, patients with chronic lung disease often develop airway obstructive disease that requires treatment with inhaled corticosteroids and other asthma medications.

In pregnant patients with CVID and lung disease, the pulmonary deficit is often exacerbated in the third trimester. If the mother receives adequate IVIG replacement therapy during pregnancy, her neonate (with CVID) has IgG levels in the reference range because the antibody is actively transported across the placenta.

Patients and their families may benefit from a periodical health-related quality-of-life assessment, highlighting the value of psychological support.
[ 41 ]

Inpatient care may be necessary, depending on the severity of the clinical manifestations secondary to CVID.

Next:

Surgical Care

Surgery is required to treat the complications of common variable immunodeficiency (CVID). Chronic sinusitis may require endoscopic sinus surgery. Severe autoimmune thrombocytopenia or hemolytic anemia can be treated with splenectomy. Biopsy should be considered to exclude infection or malignancy in enlarging lymph nodes.

Previous
Next:

Consultations

A specialist should be consulted whenever necessary.

Previous
Next:

Prevention

Because at least some CVID patients can produce protective antibody titers, one should consider the inclusion of polysaccharide vaccine in an immunization program for them.
[ 42 ]
Most CVID patients would benefit from seasonal influenza vaccination.
[ 43 ]

Previous
Next:

Long-Term Monitoring

The mainstay of outpatient care is the prevention of secondary medical conditions. IVIG should be administered every 2-4 weeks to keep the level of serum antibodies in the reference range.

Although long-term intravenous access is often required for IVIG therapy, it is not recommended. The use of a plastic cannula should be avoided because it can increase the risk of venous sclerosis. Butterfly needles should be used in their place. Alternatives to implantable venous access devices should be sought, although such devices have been used for long periods without problems.

Patients should see their physicians annually, unless they develop associated infections, which warrant immediate treatment. Physicians should obtain a thorough history and perform a thorough physical examination. Patients should be evaluated for associated conditions such infection, autoimmune disease, and malignancy.

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Medication
 

 
References

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  26. Chapel H, Lucas M, Lee M, et al. Common variable immunodeficiency disorders: division into distinct clinical phenotypes. Blood. 2008 Jul 15. 112(2):277-86. [Medline] .

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  28. Aghamohammadi A, Farhoudi A, Moin M, et al. Clinical and immunological features of 65 Iranian patients with common variable immunodeficiency. Clin Diagn Lab Immunol. 2005 Jul. 12(7):825-32. [Medline] .

  29. Kwong JC, Ward PB, Johnson PD. Cutaneous protothecosis in a patient with hypogammaglobulinemia. Med Mycol Case Rep. 2013 Jun 20. 2:132-3. [Medline] . [Full Text] .

  30. Le Cleach L, Benchikhi H, Liedman D, Boumsel L, Wolkenstein P, Revuz J. [Hand-foot-mouth syndrome recurring during common variable deficiency]. Ann Dermatol Venereol. 1999 Mar. 126(3):251-3. [Medline] .

  31. Khodadad A, Aghamohammadi A, Parvaneh N, et al. Gastrointestinal manifestations in patients with common variable immunodeficiency. Dig Dis Sci. 2007 Nov. 52(11):2977-83. [Medline] .

  32. Lassoued K. [Humoral immune deficits and the skin]. Ann Dermatol Venereol. 1999 Mar. 126(3):215-22. [Medline] .

  33. Boonyaleepun S, Boonyaleepun C, Schlactus JL. Effect of IVIG on the hair regrowth in a common variable immune deficiency patient with alopecia universalis. Asian Pac J Allergy Immunol. 1999 Mar. 17(1):59-62. [Medline] .

  34. Kilic S, Ersoy F, Sanal O, Turkbay D, Tezcan I. Alopecia universalis in a patient with common variable immunodeficiency. Pediatr Dermatol. 1999 Jul-Aug. 16(4):305-7. [Medline] .

  35. Sanjuán Álvarez M, Sánchez Zamora P, González Salvador Y, García Rueda A, Herrero Trujillano M, Rodríguez Bertos C. [Necrotising fasciitis in a patient with common variable immunodeficiency.]. Rev Esp Anestesiol Reanim. 2012 May 8. [Medline] .

  36. Pujol RM, Nadal C, Taberner R, Diaz C, Miralles J, Alomar A. Cutaneous granulomatous lesions in common variable immunodeficiency: complete resolution after intravenous immunoglobulins. Dermatology. 1999. 198(2):156-8. [Medline] .

  37. Green HA, Moschella S. Multiple invasive squamous cell carcinomas and common variable immunodeficiency. Arch Dermatol. 1992 Mar. 128(3):412-3. [Medline] .

  38. Creamer D, McGregor JM, Hawk JL. Polymorphic light eruption occurring in common variable hypogammaglobulinaemia, and resolving with intravenous immunoglobulin therapy. Clin Exp Dermatol. 1999 Jul. 24(4):273-4. [Medline] .

  39. Modrzewska K, Wiatr E, Langfort R, Oniszh K, Roszkowski-Sliz K. [Common variable immunodeficiency in a patient with suspected sarcoidosis]. Pneumonol Alergol Pol. 2009. 77(1):91-6. [Medline] .

  40. Lawrence MG, Palacios-Kibler TV, Workman LJ, Schuyler AJ, Steinke JW, Payne SC, et al. Low Serum IgE Is a Sensitive and Specific Marker for Common Variable Immunodeficiency (CVID). J Clin Immunol. 2018 Feb 17. [Medline] .

  41. Quinti I, Di Pietro C, Martini H, Pesce AM, Lombardi F, Baumghartner M, et al. Health related quality of life in common variable immunodeficiency. Yonsei Med J. 2012 May 1. 53(3):603-10. [Medline] .

  42. Rezaei N, Siadat SD, Aghamohammadi A, et al. Serum Bactericidal Antibody Response One Year after Meningococcal Polysaccharide Vaccination in Patients with Common Variable Immunodeficiency. Clin Vaccine Immunol. 2010 Jan 27. [Medline] . [Full Text] .

  43. Hanitsch LG, Löbel M, Mieves JF, Bauer S, Babel N, Schweiger B, et al. Cellular and humoral influenza-specific immune response upon vaccination in patients with common variable immunodeficiency and unclassified antibody deficiency. Vaccine. 2016 May 5. 34 (21):2417-23. [Medline] .

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  45. Shabbat S, Aharoni J, Sarid L, Ben-Harush M, Kapelushnik J. Rituximab as monotherapy and in addition to reduced CHOP in children with primary immunodeficiency and non-Hodgkin lymphoma. Pediatr Blood Cancer. 2009 May. 52(5):664-6. [Medline] .

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Media Gallery
  • Intravenous immunoglobulin infusion. Courtesy of Wikimedia Commons (Steve Brew, own work).

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    Contributor Information and Disclosures

    Author

    Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

    Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha , New York Academy of Medicine , American Academy of Dermatology , American College of Physicians , Sigma Xi

    Disclosure: Nothing to disclose.

    Coauthor(s)

    Rohit M Modak, MD, MBA Staff Physician, Department of Infectious Diseases, Virginia Hospital Center

    Disclosure: Nothing to disclose.

    Prema Modak, MD Physician, The Eye Center, Inc., Rockville, MD

    Prema Modak, MD is a member of the following medical societies: American Academy of Ophthalmology

    Disclosure: Nothing to disclose.

    Specialty Editor Board

    David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

    David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha , Association of Military Dermatologists , American Academy of Dermatology , American Society for MOHS Surgery , Phi Beta Kappa

    Disclosure: Nothing to disclose.

    Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

    Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha , American Academy of Dermatology , American College of Physicians , American College of Rheumatology

    Disclosure: Received income in an amount equal to or greater than $250 from: Lilly; Amgen <br/>Received honoraria from UpToDate for author/editor; Received honoraria from JAMA Dermatology for associate editor; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for i inherited these trust accounts; for: Allergen; Celgene; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen.

    Chief Editor

    Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

    Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

    Disclosure: Nothing to disclose.

    Additional Contributors

    Takeji Nishikawa, MD Emeritus Professor, Department of Dermatology, Keio University School of Medicine; Director, Samoncho Dermatology Clinic; Managing Director, The Waksman Foundation of Japan Inc

    Disclosure: Nothing to disclose.

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    • Sections
      Common Variable Immunodeficiency
    • Overview
      • Practice Essentials
      • Background
      • Pathophysiology
      • Etiology
      • Epidemiology
      • Prognosis
      • Patient Education
      • Show All
    • Presentation
      • History
      • Physical Examination
      • Complications
      • Show All
    • DDx
    • Workup
      • Laboratory Studies
      • Imaging Studies
      • Other Tests
      • Procedures
      • Histologic Findings
      • Staging
      • Show All
    • Treatment
      • Medical Care
      • Surgical Care
      • Consultations
      • Prevention
      • Long-Term Monitoring
      • Show All
    • Medication
      • Medication Summary
      • Immunoglobulins
      • Show All
    • Questions & Answers
    • References

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