[CIS PIDD] [cis-pidd] selective IgM deficiency

[CIS-PIDD]

Its not vasculitis? whats the lung pathologic analysis?

2017-05-11 16:50 GMT-03:00 CIS-PIDD <cis-pidd at lists.clinimmsoc.org>:

> Dear colleagues,
>
>  I am Patricia Roessler, a chilean Clinical Immunologist. .
> I would like your opinion about a clinical case, if it is possible.
>
> The patient is a male of 50 years, previously healthy until 2015 when he
> had a mild influenza H1N1 infection. Then on, he had have 6 episodes of
> pneumonia without fever but with leucocytosis, neutrophilia and elevated
> ESR and CRP. He was treated with oral antibiotics with regular response so
> he needed intravenous antibiotics to recover from this pneumonias.
> The infections were always in the same lobe ( inferior right lobe). He had
> a Fibrobronchoscopy with BAL in November 2016. There was hypercellularity
> with 95% of neutrophils and Haemphylus Influenzae grew in cultures. The
> biopsy was inespecific.
> In Lung Images no anatomic defect could be detected but as the pneumonias
> were always in the same place a lobectomy was done in March 2017.
> In Abril 2017 he had a pneumonia, now in the left lung, with fever and
> similar characteristics in general labs than previous episodes.
>
> His infectious history was anodyne, except for recurrent tonsillitis in
> infancy ( he went a tonsillectomy). He refers no sinus infections, although
> in a a PET CT done in this hospitalization a sinusitis was described.
>
> He had a gastric by-pass surgery because of obesity and diabetes in 2012 (
> both of this conditions reverted until now)
>
> He has frecuent diarrheas last year.
>
> In Immunologic studies he has:
>
> November 2016:
> IgG 1233
> IgA 436 (mild elevation)
> IgM 31 (low)
>
> Normal IgG subclasses
>
> 80% of anti pneumococcal antibodies were in protective titles (never
> vaccinated)
>
> April 2017:
> IgG 919
> IgM 22
> IgA 327
>
> Lymphocytes subpopulations were all normal.
>
> As an IgM deficiency was found he went an hematologic evaluation with
> serum electrophoresis and immunofixation being normal, and a PET CT without
> signs of an occult neoplasm. The patient denied a Bone Marrow biopsy.
>
> A malabsortion syndrome was diagnosed (low carotene, very low D vitamins
> and zinc levels, normal albumin ) and assumed to be secondary to his by
> pass.
> Celiac disease serology is negative
>
> I think that this patient has a primary selective IgM deficiency but I
> don´t know well how to treat him.
> - Do you think he needs more immunologic studies ( B memory cells and anti
> Haemophylus influenza antibodies are in course, antibodies to protein
> antigens have not been measured).
>
> - Do you think he can benefit from IVIG? (I have read that patients with
> selective IgM deficiency and low anti pneumococcal antibodies can have a
> good responde but is not the case of this patient)
>
> - Do you think that Pentaglobin could help (I have no experience with this
> IVIG)?
>
> Meanwhile he is receiving conjugated pneumococcal an H. Influenza vaccines
> and I have put him in prophylactic antibiotics ( daily
> sulphametoxazol/thrimetropin)
>
>
> Thank you very much and I hope you can help me
>
> Best regards,
>
> Patricia
>
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-- 

Leonardo Oliveira Mendonça
Médico Especialista em Imunologia Clínica e Alergia, Doenças Autoimunes e
Autoinflamatórias
Médico Especialista em Clínica Médica/Medicina Interna

Leonardo Oliveira Mendonça, MD
Specialist in Clinical Immunology and Allergy, Autoimmune and
Autoinflammatory disorders
Consultant Specialist in Internal Medicine

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