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Plasmaphresis therapy for pulmonary hemorrhage in a pediatric patient with IgA nephropathy.

Yim DK, Lee ST, Cho H - Korean J Pediatr (2015)

Bottom Line: It is an uncommon cause of end-stage renal failure in childhood.Pulmonary hemorrhage associated with IgA nephropathy is an unusual life-threatening manifestation in pediatric patients and is usually treated with aggressive immunosuppression.Pulmonary hemorrhage and renal failure usually occur concurrently, and the pulmonary manifestation is believed to be caused by the same immune process.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Samsung Medical Center, Seoul, Korea.

ABSTRACT
IgA nephropathy usually presents as asymptomatic microscopic hematuria or proteinuria or episodic gross hematuria after upper respiratory infection. It is an uncommon cause of end-stage renal failure in childhood. Pulmonary hemorrhage associated with IgA nephropathy is an unusual life-threatening manifestation in pediatric patients and is usually treated with aggressive immunosuppression. Pulmonary hemorrhage and renal failure usually occur concurrently, and the pulmonary manifestation is believed to be caused by the same immune process. We present the case of a 14-year-old patient with IgA nephropathy who had already progressed to end-stage renal failure in spite of immunosuppression and presented with pulmonary hemorrhage during oral prednisone treatment. His lung disease was comparable to diffuse alveolar hemorrhage and was successfully treated with plasmapheresis followed by oral prednisone. This case suggests that pulmonary hemorrhage may develop independently of renal manifestation, and that plasmapheresis should be considered as adjunctive therapy to immunosuppressive medication for treating IgA nephropathy with pulmonary hemorrhage.

No MeSH data available.


Related in: MedlinePlus

High-resolution computed tomography scan of the chest. The findings demonstrated multiple opacities and ground-glass attenuation in both lower lobe.
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Figure 2: High-resolution computed tomography scan of the chest. The findings demonstrated multiple opacities and ground-glass attenuation in both lower lobe.

Mentions: Two months after admission, hemoptysis and progressive dyspnea suddenly developed with oral prednisolone and cyclophosphamide. He was clinically euvolemic and no skin lesion was seen. Auscultatory examination of the chest revealed bilateral coarse crepitations. Chest x-ray showed bilateral pulmonary infiltration and chest computed tomography showed pulmonary hemorrhagic lesions on both lower lung fields (Fig. 2). He had no history of pulmonary symptoms or reported abnormalities on chest x-ray. Our patient was a nonsmoker with no known exposure to nonprescribed medication. There were no gastrointestinal, joint or skin symptoms. Extensive microbiologic screening results were all negative. In the laboratory findings, there was no risk of bleeding tendency. A prothrombin time international normalized ratio was 1.02, activated partial thromboplastin time 38.7 seconds, and platelet counts 130,000/mm3. With the peritoneal dialysis, urine protein was 1.3 g/day, and glomerular filtration rate 3.1 mL/min/1.73 m2. Repeated tests for serum antinuclear antibody, antiglomerular basement membrane antibody, and ANCA were also all negative. Pulse oximetry was below 90% on a FiO2 of 40%. He was moved to the intensive care unit due to hypoxia, which was treated with mask oxygen supplementation. He received continuous renal replacement therapy (CRRT) and intravenous antibiotics and antifungal agent. However, there was no clinical response to the antibiotics, antifungal agent or CRRT. Heavy hemosiderin-laden macrophages were seen by bronchoscopy. Cultures from the bronchoalveolar lavage were negative for microorganisms. This finding was suggestive of diffuse alveolar hemorrhage. The clinical manifestations and bronchoscopy findings suggested a pulmonary renal syndrome secondary to IgA nephropathy. The patient received daily plasmapheresis with albumin and intravenous methylprednisolone at 1 mg/kg/day and the respiratory symptoms and hypoxia showed rapid resolution after 3 plasmapheresis treatments. The chest x-ray findings were also improved. The patient was discharged with an oral prednisolone dose of 0.5 mg/kg/day and weekly plasmapheresis. The prednisolone and plasmapheresis were gradually tapered and stopped over 3 months. There was no relapse over the proceeding 8 months of follow-up.


Plasmaphresis therapy for pulmonary hemorrhage in a pediatric patient with IgA nephropathy.

Yim DK, Lee ST, Cho H - Korean J Pediatr (2015)

High-resolution computed tomography scan of the chest. The findings demonstrated multiple opacities and ground-glass attenuation in both lower lobe.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4644770&req=5

Figure 2: High-resolution computed tomography scan of the chest. The findings demonstrated multiple opacities and ground-glass attenuation in both lower lobe.
Mentions: Two months after admission, hemoptysis and progressive dyspnea suddenly developed with oral prednisolone and cyclophosphamide. He was clinically euvolemic and no skin lesion was seen. Auscultatory examination of the chest revealed bilateral coarse crepitations. Chest x-ray showed bilateral pulmonary infiltration and chest computed tomography showed pulmonary hemorrhagic lesions on both lower lung fields (Fig. 2). He had no history of pulmonary symptoms or reported abnormalities on chest x-ray. Our patient was a nonsmoker with no known exposure to nonprescribed medication. There were no gastrointestinal, joint or skin symptoms. Extensive microbiologic screening results were all negative. In the laboratory findings, there was no risk of bleeding tendency. A prothrombin time international normalized ratio was 1.02, activated partial thromboplastin time 38.7 seconds, and platelet counts 130,000/mm3. With the peritoneal dialysis, urine protein was 1.3 g/day, and glomerular filtration rate 3.1 mL/min/1.73 m2. Repeated tests for serum antinuclear antibody, antiglomerular basement membrane antibody, and ANCA were also all negative. Pulse oximetry was below 90% on a FiO2 of 40%. He was moved to the intensive care unit due to hypoxia, which was treated with mask oxygen supplementation. He received continuous renal replacement therapy (CRRT) and intravenous antibiotics and antifungal agent. However, there was no clinical response to the antibiotics, antifungal agent or CRRT. Heavy hemosiderin-laden macrophages were seen by bronchoscopy. Cultures from the bronchoalveolar lavage were negative for microorganisms. This finding was suggestive of diffuse alveolar hemorrhage. The clinical manifestations and bronchoscopy findings suggested a pulmonary renal syndrome secondary to IgA nephropathy. The patient received daily plasmapheresis with albumin and intravenous methylprednisolone at 1 mg/kg/day and the respiratory symptoms and hypoxia showed rapid resolution after 3 plasmapheresis treatments. The chest x-ray findings were also improved. The patient was discharged with an oral prednisolone dose of 0.5 mg/kg/day and weekly plasmapheresis. The prednisolone and plasmapheresis were gradually tapered and stopped over 3 months. There was no relapse over the proceeding 8 months of follow-up.

Bottom Line: It is an uncommon cause of end-stage renal failure in childhood.Pulmonary hemorrhage associated with IgA nephropathy is an unusual life-threatening manifestation in pediatric patients and is usually treated with aggressive immunosuppression.Pulmonary hemorrhage and renal failure usually occur concurrently, and the pulmonary manifestation is believed to be caused by the same immune process.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Samsung Medical Center, Seoul, Korea.

ABSTRACT
IgA nephropathy usually presents as asymptomatic microscopic hematuria or proteinuria or episodic gross hematuria after upper respiratory infection. It is an uncommon cause of end-stage renal failure in childhood. Pulmonary hemorrhage associated with IgA nephropathy is an unusual life-threatening manifestation in pediatric patients and is usually treated with aggressive immunosuppression. Pulmonary hemorrhage and renal failure usually occur concurrently, and the pulmonary manifestation is believed to be caused by the same immune process. We present the case of a 14-year-old patient with IgA nephropathy who had already progressed to end-stage renal failure in spite of immunosuppression and presented with pulmonary hemorrhage during oral prednisone treatment. His lung disease was comparable to diffuse alveolar hemorrhage and was successfully treated with plasmapheresis followed by oral prednisone. This case suggests that pulmonary hemorrhage may develop independently of renal manifestation, and that plasmapheresis should be considered as adjunctive therapy to immunosuppressive medication for treating IgA nephropathy with pulmonary hemorrhage.

No MeSH data available.


Related in: MedlinePlus