Dr Erwa N. H. H.1, 2, Dr Al Hussain H. A. 2Dr A Aziz S. I. 1, 2 & Prof Ahmed A. H.1
1 Faculty of Medicine University of Khartoum, Khartoum Sudan
2 Soba University Hospital, university of Khartoum, Khartoum, Sudan

Common Variable Immunodeficiency (CVID) represents a heterogeneous group of primary antibody deficiencies (PADs) which is increasingly becoming a diagnosis of exclusion. The diagnosis is based on a clinical presentation of repeated infections mainly of the respiratory tract and decreased immunoglobulins of the IgG, IgA with or without a decrease in the IgM isotype1,2. Infections are usually bacterial and viral whereas fungal infections are unusual as primary pathogens in patients with primary antibody deficiency unless there is a substantial T cell defect 2, 3. While many immunological defects have been reported in CVID patients, primary Aspergillus infection is not commonly seen in this group of patients 3.

Case:

A 38 year old male was referred by the chest physician September 2014 because of repeated sinupulmonary infections. His problems started in 2000 with sinus problems, repeated chest infections including pneumonias and 2 previous bouts of, what he was told were attacks of, viral meningitis/meningoencephalitis and from which he recovered completely. He was tired most of the time and had a reduced appetite. His knees and elbows were painful with swellings of the knees which have been investigated by the rheumatologists and for which he was given steroids without benefit. He had, repeatedly, tested negative for HIV and a bone marrow examination was non conclusive. Sinuses CT revealed obliteration of all sinuses more marked on the right side. He had to quit his job.

On examination he looked unwell, thin and slightly pale. There was no significant lymphadenopathy. Examination of the chest revealed areas of bronchial breathing mainly on the right middle and lower lobes.

He had a normal haemoglobin, white cell count and differential with normal renal and liver profiles, normal CRP and normal C3&C4.

Immunoglobulins:   IgG: 79 mg/dl (700-1600)

IgA: 7 mg/dl (70-400)

IgM: 13mg/dl (40-230)

A diagnosis of CVID was suggested and the patient was started on Doxycycline for 4 weeks to cover for a possible Mycoplasma joint infection. He was then prescribed Cotrimoxazole (Septrin) prophylaxis till he arranged financial support for immunoglobulin replacement. Unfortunately the Septrin was not commenced. In the first week of December 2014, he became very ill with an acute onset of right sided chest pain following an upper respiratory tract infection for which he took Azithromycin. He was very tired, pale and had diarrhea. Examination showed severe right sided tenderness on the lower chest wall and hypocondrium with a degree of guarding on the same region. His white cell count was raised with neutrophilia and a raised CRP. A chest X ray confirmed a right sided pneumonia with a pneumonic effusion. Abdominal ultrasound revealed a normal liver texture with no evidence of abscess formation and a slightly enlarged spleen. HRCT scan of the chest showed right middle lobar pneumonia with para-pneumonic effusion, minimal bronchial wall thickening of the upper and middle lobes, small pre-vascular lymph nodes and no abnormality of the mediastinum. Lymphocyte subsets (T/B/NK) analysis revealed that his B lymphocytes were 2.97% , NK cells 5.4% , T lymphocytes were 89.5%.

Sputum examination showed the presence of Klebsiella pneumonia and Aspergillus sp. Stool culture grew Salmonella species which was sensitive to Ciprofloxacin to which the Klebsiella was also sensitive. He was admitted to hospital for fluid and electrolytes correction and was started on I/V Ciprofloxacin.  His conditioned improved and was discharged on oral antibiotics.

Discussion:

Four to 9 years of delay in the diagnosis of CVID have been reported1. It is not unusual to encounter longer delays in countries with minimal reporting of PIDs. This might worsen the prognosis which is further compounded by limited diagnostic and treatment facilities 1, 4.

As Aspergillus sp infections is not common in patients with PAD, further samples have been sent for mycology analysis. We hypothesize some explanations in order of importance: secondary infection due to chronic sinusitis, contaminant colonies, or an unusual presentation of CVID.

Conclusions:

This scenario demonstrates diagnostic delays in countries with limited resources and an unusual pathogen encountered in PAD.

References:

  1. Salzer U., Warnatz K. & Peter H. H. Common Variable Immunodeficiency- an update. Arthritis Research & Therapy. 2012, 14:223.
  2. Bousfiha A.A., et al. A phenotypic approach for IUIS PID classification and diagnosis. Guidelines for clinicians at the bedside. J Clin Immunol. 2013 August;33 (6): 1078-1087.
  3. Antachopoulos C., Walsh T. J. & Roilides E. Fungal infections in primary immunodeficiencies. Euro J Pediatr (2007) 116: 1099-1117.
  4. Sorensen R., Etzioni A., Bousfiha A. A. & Zeiger J. B. Collaborating to improve quality of life in primary immunodeficiencies: World PI Week, 2013. J Clin Immunol (2013) 33:1145-1148.

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