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Hi Nutty,

Firstly - good to meet you!

And I'm very sorry to read about your sister and her CVID and the hepatitis. It must have been awful for your family.

I was diagnosed with SLE and APS in June 2004, I already had low immunoglobulins G and A then. But I only got a diagnosis of CVID in May of this year. That is because I finally got to the right centre of excellence for primary immunodeficiency in London (royal free hospital). They have recommended I start the IVIG treatment - which is immunoglobulin by IV as I guess your nephew has. My lupus doc also thinks this IVIG will help my quite bad lupus.

It was very interesting at the immunodeficiency appointment. I have only had one appt so far - next one is next week. The consultant who saw me said that SLE and CVID are linked. CVID is a rare complication of lupus where people have had severe lupus as their immune systems go into 'early retirement'. This means they are no longer producing all the antibodies (or not so many). My lupus doctor who is at St Thomas' hospital also tells me it's linked but rare . . . . . . he only has one other patient who has a subclass deficiency and lupus but he says there are other cases around the world.

Whilst searching myself I found an article on the internet by someone in Spain whos found a link between SLE and CVID. . . . . I will try and link it for you on the next post when I've worked out how to do it.

Please ask me any other questions - I too really want to work out the link - afterall it might help us and others work out what is really going wrong with our immune systems causing all these problems

Cathy x
 

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Management of hypogammaglobulinaemia occurring in patients with systemic lupus erythematosus

P. F. K. Yong1,2, L. Aslam2, M. Y. Karim2,3 and M. A. Khamashta2

[SIZE=-1]1Department of Clinical Immunology, Kings College Hospital, 2Louise Coote Lupus Unit, St Thomas’ Hospital, London and 3Department of Immunology, Frimley Park Hospital, Frimley, UK. [/SIZE]

[SIZE=-1]
Correspondence to: M. Y. Karim, Louise Coote Lupus Unit, St Thomas’ Hospital, Lambeth Palace Road, London SE1 7EH, UK. E-mail: [email protected] [/SIZE]

[SIZE=+2] Abstract[/SIZE]
Objectives. Systemic lupus erythematosus (SLE) is typically associated with hypergammaglobulinaemia but has been described in the setting of hypogammaglobulinaemia as well. The purpose of this article is to describe various cases of SLE and hypogammaglobulinaemia, review the literature and present management strategies for hypogammaglobulinaemia in SLE.
Methods. We describe five patients with SLE and antibody deficiency, and review the literature exploring the relationship between the two.
Results. Various types of antibody deficiency syndromes, including common variable immunodeficiency (CVID), IgA deficiency, IgM deficiency, drug-induced hypogammaglobulinaemia and hypogammaglobulinaemia secondary to nephrotic syndrome can occur in SLE. Antibody deficiency states can be treated with antibiotics and replacement immunoglobulin therapy (particularly CVID) but sometimes close monitoring is all that is required. Conclusion. Measurement of immunoglobulin levels is useful in SLE to identify coexisting antibody deficiency and the later development of hypogammaglobulinaemia. This allows monitoring and appropriate treatment to be instituted.
 

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MADRID, Spain—Systemic lupus erythematosus (SLE) and common variable immunodeficiency (CVID) are at opposite poles, immunologically speaking, but a growing number of CVID cases is being reported in lupus patients. Because SLE develops first, rheumatologists should monitor immunoglobulin profiles in lupus patients and should consider CVID in all lupus patients who have recurrent sinopulmonary infections in the absence of lupus activity and/or immunosuppressive treatment, according to a new study in the February issue of Seminars in Arthritis & Rheumatism.1

"The findings of an apparent association between these two disorders in this small group of patients should alert physicians to a new long-term consequence of lupus" —Mónica Fernández-Castro, MD, of the Hospital Universitario Puerta de Hierro, Madrid, Spain.
"Recurrent infections in a patient with SLE activity, apart from SLE activity and/or immunosuppresive treatment, must alert the physician to possible CVID and treatment with gammaglobulins may be highly effective," conclude researchers led by Mónica Fernández-Castro, MD, of the Hospital Universitario Puerta de Hierro, Madrid, Spain.
Two polar extremes of immune reactivity
Dr. Fernández-Castro and colleagues described the cases of two patients with SLE and CVID and identified 16 other patients through a literature review. Overall, 89% of CVID patients were females who had a mean age of 23.8 years at the onset of SLE. CVID developed within the first 5 years after the lupus diagnosis in half these patients, the study showed. All patients had been treated with corticosteroids and 72% had also received immunosuppressive therapy. The most frequent symptom was sinopulmonary infections. SLE disease activity decreased after the development of CVID in 67% of patients. Most patients (89%) were treated with gammaglobulin therapy. The most notable immunological feature was a reduced number or percentage of B-cells in 60% of patients.
CVID in lupus may be caused by an intrinsic B-cell deficit or by an extrinsic factor with an effect on B-cell maturation, they hypothesize. Whether these two seemingly opposite disorders coexist or if the CVID is a complication related to the SLE or SLE treatment is not fully understood.

"The findings of an apparent association between these two disorders in this small group of patients should alert physicians to a new long-term consequence of lupus," study authors conclude.
Telltale signs of CVID in lupus
"Although SLE-associated CVID is uncommon, due to its potentially fatal outcome, it should be considered in any SLE patient with hypogammaglobulinemia (at least 2 standard deviations below the mean for age in serum concentration of IgG and IgA), poor or absent response to immunization (two-fold or less increase in antibody titer), and acute, chronic, or recurrent infections, specifically pneumonia bronchitis, sinusitis conjunctivitis, and otitis," Dr. Fernández-Castro writes.
Because all of the identified patients were diagnosed with lupus before CVID, the authors call for immunoglubulin measurements in SLE patients.
Measure immunoglubulin before chemo
"CVID in lupus is a rare thing—even in my population," said Robert Lahita MD, PhD, FACP, FACR, FRCP, professor of medicine at Mount Sinai Medical School, in New York, and chairman of medicine at the Jersey City Medical Center, in New Jersey. A lupus specialist, Dr. Lahita, the editor of Systemic Lupus Erythematosus, added "If you have chronic sinusitis or some suggestion of immunodeficiency, you should be tested for immunoglubulin levels."
Dr. Lahita said that currently he only runs a panel of immunoglubulins on patients in whom he is unable to make a straightforward lupus diagnosis.
"I probably would still treat the SLE patient the same way," he said. "If you are intending to give a lupus patient tremendous amounts of chemotherapy like cyclophosphamide [Cytoxan®, Neosar®] I think you should test for immunodeficiency. If you give Cytoxan and knock out bone marrow, you are going to also enhance the patient's risk of getting infected, since they don't have much immunoglubulin lying around."
References
1. Fernández-Castro M, Mellor-Pita S, Citores MJ, et al. Common variable immunodeficiency in systemic lupus erythematosus. Semin Arthritis Rheum. 2007;36:238-245.

http://www.mskreport.com/articles.cfm?articleID=1188
 

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Hi Nutty

Hope these two articles I have found hellp - the first one from london I have not seen before so I'll be interested to try and find the full article of that one.

I hope they help others too. Afterall, although it's clearly 'rare' to have CVID and SLE it's not unheard of, so it's something that we should all be aware of, and get our docs to check us out for , if we're concerned

cathy x
 
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