Severe Aspergillus Pneumonia and Pulmonary Artery Hypertension in a Child with Autosomal Recessive Chronic Granulomatous Disease and Selective IgA Deficiency


To the editor Chronic granulomatous disease (CGD) is a phagocyte disorder characterized by a defect in the neutrophil respiratory burst that predisposes to recurrent infections with catalase-positive microorganisms [1, 2]. In addition, these patients have inflammatory complications predominantly involving the gastrointestinal tract, lungs, and urinary tract [3, 4]. It is difficult to differentiate clinically the inflammatory lung disease from bacterial or fungal pneumonia. Selective IgA deficiency is the most common immunodeficiency disorder and it may also produce autoimmune manifestations [5, 6]. Co-occurrence of CGD and selective IgA deficiency is rare and seldom reported in literature. A 7-year-old boy, born of a non-consanguineous marriage, presented to us with a history of a cough, respiratory distress, and fever for 1 month. He had had two hospitalizations during the last 1 month for these complaints. In the past, at 6 years of age, he had an episode of dysentery, for which oral antimicrobials were prescribed. Three of his male siblings had died (at 3, 7, and 5 years of age, respectively) because of an unexplained acute respiratory illness. On examination, he had pallor, grade 2 clubbing and crepitations on auscultation of the chest. Rest of the examination was normal. Laboratory investigations revealed hemoglobin 112 g/L, white blood cell count 19 × 10 cells/L (differential count polymorphs 70%, lymphocytes 28%, and monocytes 2%), platelet counts 411 × 10/L, alanine aminotransferase (ALT) 414 U/L, aspartate aminotransferase (AST) 484 U/L, and human immunodeficiency virus (HIV) serology by enzyme-linked immunosorbent assay (ELISA) was non-reactive. Immunoglobulin profile revealed IgG 1710 mg/dL (normal range 540–1610), IgA <17 mg/dL (normal range 50–240), and IgM 141 mg/dL (normal range 50–180). Lymphocyte subset analysis revealed CD3+ T cells 70%, CD3+ CD4+ T cells 14%, CD19+ B cells 13%, and CD16+/56+ natural killer cells 6.3%. Nitroblue tetrazolium dye reduction test revealed no reduction of NBT dye by stimulated neutrophils and dihydrorhodamine 123 assay revealed markedly reduced stimulation index in the patient neutrophils as compared to control (1.08 in the patient and 159.5 in the control). Flow cytometric expression of the b558 protein on neutrophils was normal. DNA sequencing of the NCF-1 gene revealed a homozygous GT deletion in the exon 2 (Supplementary figure). Chest X-ray revealed ground-glass opacities in bilateral lung fields (Fig. 1a) and contrastenhanced computerized tomography (CECT) scan of the chest revealed diffuse ground-glass opacities, septal thickening, nodules, interspersed cysts, and dilatedmain pulmonary artery and right ventricle (Fig. 1b–d). Blood culture was sterile and culture from tracheal aspirate grew Aspergillus flavus. Gastric lavage for acid-fast bacilli (AFB) stain revealed no acid-fast bacilli and tuberculin skin test was non-reactive. Antinuclear antibody (ANA) test showed 2+ speckled pattern (on indirect immunofluorescence at 1:40 dilution) and anti-doubles t r a n d e d DNA an t i b o d y t i t e r w a s 4 0 IU /mL All authors have contributed equally to the preparation and editing of this manuscript.

DOI: 10.1007/s10875-017-0386-6

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@article{Jindal2017SevereAP, title={Severe Aspergillus Pneumonia and Pulmonary Artery Hypertension in a Child with Autosomal Recessive Chronic Granulomatous Disease and Selective IgA Deficiency}, author={Ankur Kumar Jindal and Amit Rawat and Deepti Suri and Madhubala Sharma and Jitendra Kumar Shandilya and Shubham Goel and Kushaljit Singh Sodhi and Surjit Kumar Singh}, journal={Journal of Clinical Immunology}, year={2017}, volume={37}, pages={333-335} }