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Serum antibody-negative Goodpasture syndrome with delta granule pool storage deficiency and eosinophilia.

Kussman A, Gohara A - Clin Kidney J (2012)

Bottom Line: After a 14-day hospital stay and extensive workup, as well as treatment with antibiotics, steroids and ventilator support for respiratory failure, the patient continued to deteriorate and entered multisystem organ failure.The family decided to withdraw ventilator support, and the patient expired.Immunofluorescence testing for anti-GBM autoantibodies on lung and kidney tissues during an autopsy confirmed the diagnosis of Goodpasture syndrome.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology , University of Toledo Medical Center , Toledo, OH , USA.

ABSTRACT
Goodpasture syndrome is a rare, life-threatening autoimmune disease characterized by a triad of rapidly progressive glomerulonephritis, a hemorrhagic pulmonary condition and the presence of anti-glomerular basement membrane (anti-GBM) antibodies. The antibodies initiate destruction of the kidney glomeruli, resulting in a focal necrotizing glomerulitis, which may progress rapidly to renal failure. Autoantibody-mediated damage of alveolar basement membranes leads to diffuse pulmonary hemorrhage, which in some cases may be severe enough to cause respiratory failure. Many clinicians use a variety of assays to detect serum anti-GBM antibodies; however, these tests may be falsely negative in up to 15% of patients with Goodpasture syndrome. Here, we report an unusual case of a 40-year-old man with clinical evidence of Goodpasture syndrome, a negative anti-GBM antibody serum result, eosinophilia and delta granule pool storage deficiency. After a 14-day hospital stay and extensive workup, as well as treatment with antibiotics, steroids and ventilator support for respiratory failure, the patient continued to deteriorate and entered multisystem organ failure. The family decided to withdraw ventilator support, and the patient expired. Immunofluorescence testing for anti-GBM autoantibodies on lung and kidney tissues during an autopsy confirmed the diagnosis of Goodpasture syndrome.

No MeSH data available.


Related in: MedlinePlus

Photomicrograph of linear IgG deposits along the GBM of a kidney tissue section shown via immunofluorescence.
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SFS107F4: Photomicrograph of linear IgG deposits along the GBM of a kidney tissue section shown via immunofluorescence.

Mentions: An autopsy was performed to determine the cause of this patient's demise. The major gross autopsy finding was severe diffuse pulmonary hemorrhage. Significant microscopic findings included those in the lungs and kidneys. Sections of the lungs revealed extensive intra-alveolar hemorrhage, edema, hemosiderin-laden macrophages, focal neutrophilic infiltration, wide-spread chronic inflammation and total disruption of the normal lung architecture (Figures 1 and 2). Microscopic examination of the kidneys revealed extensive tubular cell nuclei dropout, consistent with acute tubular necrosis, which was caused by his persistent hypoxia secondary to his respiratory failure, and mesangial cell proliferation in the glomeruli, fibrin in the Bowman spaces and occasional early crescent formation secondary to inflammatory destruction of the basement membranes in the renal glomeruli. The most significant study involved immunofluorescence, which revealed focal faint linear immunoglobulin G (IgG) deposits along the alveolar septa and strong extensive peripheral linear IgG deposits in the capillary loops of the renal glomeruli (Figures 3 and 4). These findings are consistent with Goodpasture's syndrome.Fig. 1.


Serum antibody-negative Goodpasture syndrome with delta granule pool storage deficiency and eosinophilia.

Kussman A, Gohara A - Clin Kidney J (2012)

Photomicrograph of linear IgG deposits along the GBM of a kidney tissue section shown via immunofluorescence.
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4400547&req=5

SFS107F4: Photomicrograph of linear IgG deposits along the GBM of a kidney tissue section shown via immunofluorescence.
Mentions: An autopsy was performed to determine the cause of this patient's demise. The major gross autopsy finding was severe diffuse pulmonary hemorrhage. Significant microscopic findings included those in the lungs and kidneys. Sections of the lungs revealed extensive intra-alveolar hemorrhage, edema, hemosiderin-laden macrophages, focal neutrophilic infiltration, wide-spread chronic inflammation and total disruption of the normal lung architecture (Figures 1 and 2). Microscopic examination of the kidneys revealed extensive tubular cell nuclei dropout, consistent with acute tubular necrosis, which was caused by his persistent hypoxia secondary to his respiratory failure, and mesangial cell proliferation in the glomeruli, fibrin in the Bowman spaces and occasional early crescent formation secondary to inflammatory destruction of the basement membranes in the renal glomeruli. The most significant study involved immunofluorescence, which revealed focal faint linear immunoglobulin G (IgG) deposits along the alveolar septa and strong extensive peripheral linear IgG deposits in the capillary loops of the renal glomeruli (Figures 3 and 4). These findings are consistent with Goodpasture's syndrome.Fig. 1.

Bottom Line: After a 14-day hospital stay and extensive workup, as well as treatment with antibiotics, steroids and ventilator support for respiratory failure, the patient continued to deteriorate and entered multisystem organ failure.The family decided to withdraw ventilator support, and the patient expired.Immunofluorescence testing for anti-GBM autoantibodies on lung and kidney tissues during an autopsy confirmed the diagnosis of Goodpasture syndrome.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology , University of Toledo Medical Center , Toledo, OH , USA.

ABSTRACT
Goodpasture syndrome is a rare, life-threatening autoimmune disease characterized by a triad of rapidly progressive glomerulonephritis, a hemorrhagic pulmonary condition and the presence of anti-glomerular basement membrane (anti-GBM) antibodies. The antibodies initiate destruction of the kidney glomeruli, resulting in a focal necrotizing glomerulitis, which may progress rapidly to renal failure. Autoantibody-mediated damage of alveolar basement membranes leads to diffuse pulmonary hemorrhage, which in some cases may be severe enough to cause respiratory failure. Many clinicians use a variety of assays to detect serum anti-GBM antibodies; however, these tests may be falsely negative in up to 15% of patients with Goodpasture syndrome. Here, we report an unusual case of a 40-year-old man with clinical evidence of Goodpasture syndrome, a negative anti-GBM antibody serum result, eosinophilia and delta granule pool storage deficiency. After a 14-day hospital stay and extensive workup, as well as treatment with antibiotics, steroids and ventilator support for respiratory failure, the patient continued to deteriorate and entered multisystem organ failure. The family decided to withdraw ventilator support, and the patient expired. Immunofluorescence testing for anti-GBM autoantibodies on lung and kidney tissues during an autopsy confirmed the diagnosis of Goodpasture syndrome.

No MeSH data available.


Related in: MedlinePlus