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

[CIS-PIDD]

Is he aspirating?

Dr Karen Lindsay
Rheumatologist and Immunology Fellow
MBChB BSc MRCP (UK)
From: cis-pidd at lyris.dundee.net [mailto:cis-pidd at lyris.dundee.net] On Behalf Of CIS-PIDD
Sent: Friday, 12 May 2017 9:54 a.m.
To: CIS-PIDD
Subject: Re: [cis-pidd] selective IgM deficiency

Its not vasculitis? whats the lung pathologic analysis?

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