Goodpasture's syndrome with positive C-ANCA and normal renal function: A case report

Ramaswami, Arunachalam; Kandaswamy, Thiraviyam; Rajendran, Tholappan; Aung, Hla; Jacob, C. K.; Zinna, Haji Shaukat; Telesinge, Pemasiri Upali

doi:10.1186/1752-1947-2-223

Cited by 6 publications

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“…A linear pattern of immunoglobulin along the basement membrane of renal glomerular capillaries and alveolar septa seen by immunofluorescence or immunohistochemistry is consistent with Goodpasture syndrome. The latter diagnostic tests are important in suspected clinical cases in which the serum anti-GBM antibody test is negative, as up to 15% of patients with Goodpasture syndrome may have no detectable circulating antibodies [ 7 ]. This was the case in our patient, in which his repeated anti-GBM antibody tests came back negative; however, the presence of these antibodies was confirmed by immunofluorescence in lung and renal tissue sections.…”

Section: Discussionmentioning

confidence: 99%

“…Other test results included a positive rheumatoid factor, autoantibodies, weakly positive anti-double stranded deoxyribonucleic acid negative cytoplasmic-ANCA and extensive ground glass appearance on a chest computed tomography scan. Lung biopsies showed linear deposition of IgG in the alveolar capillary basement membranes using immunoperoxidase staining, and linear IgG and C3 were seen along the renal capillary walls with immunofluorescence [ 7 ]. Finally, a case of a 27-year-old male with Goodpasture syndrome and undetectable anti-GBM circulating antibodies was reported by Hellmann et al .…”

Section: Discussionmentioning

confidence: 99%

“…On the contrary, the circulating serum antibodies may be of lower affinity than those that are tissue bound and thus are more easily washed away during the wash-out step of the assay. Finally, the assay's renal tissue may have a different epitope than that present in the native kidney [ 1 , 7 ]. For patients who present promptly with hemoptysis and subsequently receive steroid therapy early in the disease process, negative anti-GBM serum results along with only focal linear deposits in tissues may be due to steroid-induced immune suppression [ 6 ].…”

Section: Discussionmentioning

confidence: 99%

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

Kussman

Gohara

2012

Clinical Kidney Journal

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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.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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

Kussman

Gohara

2012

Clinical Kidney Journal

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“…Obwohl bei pulmonaler Manifestation dieser Erkrankungen fast immer eine renale Beteiligung vorliegt, sind auch seltene Fälle des isolierten pulmonalen Befalls beschrieben [1][2][3][4][5].…”

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Das pulmorenale Syndrom auf der Intensivstation

2010

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“…The etiology of Goodpasture's Syndrome remains unknown; however, the development of the disease following environmental exposures including hydrocarbon fumes, metallic dust or tobacco smoke has been reported (5). GPS occurs due to antibody and T-lymphocyte reactivity to the NC1 domain of the α3 chain of type IV collagen (6).…”

Section: Introductionmentioning

confidence: 99%

Goodpasture's syndrome in early pregnancy: A case report

Qin¹,

Song²,

Liu³

2017

Exp Ther Med

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Abstract. Goodpasture's syndrome (GPS) presenting during pregnancy is extremely rare and patients exhibiting hemoptysis and renal dysfunction in early pregnancy are uncommon. The present study reports the case of a 17-year-old diagnosed with GPS during the thirteenth week of pregnancy. Prompt recognition and immediate treatment (steroids and plasma exchange) was initiated. Renal function normalized following treatment and anti-GBM antibody became negative during hospital stay. However, due to irregular follow-up, the patient eventually succumbed. The case highlights that the current treatment plan for GPS should be revised to improve the outcome of pregnancy. In addition, it determines how important it is for obstetricians to consider whether a pregnancy should be terminated to improve the chances of survival of pregnant patients with GPS. IntroductionGoodpasture's syndrome (GPS) is an immuno disorder characterized by pulmonary hemorrhage, glomerulonephritis and the presence of the antiglomerular basement membrane (anti-GBM) antibody (1,2). The incidence of GPS is <1 case/1,000,000 (3). Combination therapy is recommended for the initial management of the disease, including corticosteroid therapy, cytostatic therapy and plasmapheresis. However, patients have poor prognosis with current treatments and the mortality rate has been estimated as 11% (4). The etiology of Goodpasture's Syndrome remains unknown; however, the development of the disease following environmental exposures including hydrocarbon fumes, metallic dust or tobacco smoke has been reported (5). GPS occurs due to antibody and T-lymphocyte reactivity to the NC1 domain of the α3 chain of type IV collagen (6).Women presenting with GPS in the early period of pregnancy are uncommon and the present case study reports an unusual case of GPS in the early stage of pregnancy. In the present study, the patient received hemodialysis and methylprednisolone, prior to succumbing due to sustained, irreversible renal failure. The majority of pregnancies are terminated prior to term delivery to prevent worsening of conditions (7,8). Thus, current therapeutic strategies used to treat GPS should be revised in order to improve the survival rate of pregnant patients with GPS. Case reportA 17-year-old female was hospitalized at the Traditional Chinese Medicine Hospital of Guizhou (Guiyang, China) on 9th December 2013 due to no urination and hemoptysis for 3 days in the thirteenth week of pregnancy. The patient had history of double lower limb swelling lasting from the onset of pregnancy, which was treated with furosemide in Qianxi County Central hospital. The patient's medical history prior to pregnancy was normal; however, the patient had a 2-week history of cough prior to admission and experienced an episode of brown-colored urine 1 week later. The patient did not experience fever, dysuria or oliguria at that time. The patient visited Qianxi County Central hospital (Quianxi, China) on 21th October 2013 and was diagnosed with acute renal insufficiency. Serum was analysed ...

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Goodpasture's syndrome with positive C-ANCA and normal renal function: A case report

Cited by 6 publications

References 6 publications

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

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

Das pulmorenale Syndrom auf der Intensivstation

Goodpasture's syndrome in early pregnancy: A case report

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