An uncommon presentation of a rare disease: A case of anti-glomerular basement membrane disease without renal involvement

Polman, Jeremy; Panzarello, Rory; Patel, P. L.; Tati, Vasudev

doi:10.1016/j.rmcr.2020.101282

Cited by 3 publications

(7 citation statements)

References 11 publications

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“…In the present case, anti-GBM antibody was positive but had a relatively low titer. To our knowledge, there have been three cases of anti-GBM disease with isolated DAH in which antibody titers were lower than that in our case or undetectable ( 5 , 16 , 17 ). In one of the cases, the authors speculated that anti-GBM antibody existed solely in a tissue-bound form and was not detectable in the circulation ( 17 ).…”

Section: Discussioncontrasting

confidence: 76%

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Rituximab for the Treatment of Anti-glomerular Basement Membrane Disease with Isolated Diffuse Alveolar Hemorrhage

et al. 2023

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Anti-glomerular basement membrane (GBM) disease with isolated diffuse alveolar hemorrhage (DAH) is rare. We herein report a 91-year-old man admitted with hypoxia and diagnosed with anti-GBM disease with DAH based on positive bronchoalveolar lavage and serum antibody test results. There was no renal involvement. Although remission was achieved using glucocorticoids and plasmapheresis, the patient experienced DAH relapse one week after the last plasmapheresis. Rituximab 375 mg/m 2 was administered 4 times weekly; thereafter, DAH relapse was not observed, and the glucocorticoid dosage was tapered. Rituximab was thus effective in treating anti-GBM disease with isolated DAH in an extremely elderly patient.

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Section: Discussioncontrasting

confidence: 76%

“…Approximately 98.9% of patients with anti-GBM disease present with rapidly progressive glomerulonephritis, whereas 45.5% develop DAH (3). Although some studies have reported anti-GBM disease with isolated DAH in patients, this clinical manifestation accounts for only 0.99% of all anti-GBM disease cases (3)(4)(5).…”

Section: Introductionmentioning

confidence: 99%

Rituximab for the Treatment of Anti-glomerular Basement Membrane Disease with Isolated Diffuse Alveolar Hemorrhage

et al. 2023

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“…Most researchers maintain that it is unclear as to what triggers anti-GBM disease. However, some have postulated that pulmonary insults, such as an infection, smoking, inhaled hydrocarbons, and renal injury could be triggering factors [ 12 , 13 ]. The underlying bronchiectasis may have triggered the disease of Ms. X.…”

Section: Discussionmentioning

confidence: 99%

Hemoptysis of Uncertain Cause Leading to Delayed Diagnosis in an Elderly Lady With Anti-glomerular Basement Membrane Disease

Razik,

Aslam

2023

Cureus

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Anti-glomerular basement membrane (GBM) disease or Goodpasture syndrome is a rare disorder characterized by anti-GBM autoantibodies targeting the type 4 collagen of the basement membrane, resulting in rapidly progressive glomerulonephritis with or without alveolar hemorrhage. Pulmonary manifestations are less common in the elderly. Isolated pulmonary manifestations are rare in all age groups, and even more so in the elderly. We present the case of a lady in her late 70s, who presented initially with massive hemoptysis in the absence of renal dysfunction, which was presumed to be secondary to underlying bronchiectasis and infection. However, she later developed rapidly progressive acute kidney injury despite improvement in pulmonary symptoms and was diagnosed with anti-GBM disease. The delay in diagnosis and subsequent treatment due to the atypical presentation resulted in irreversible renal injury and the need for lifelong dialysis. This case demonstrates the need to consider atypical presentations of rare disorders, to ensure early diagnosis and optimal prognosis, especially when the clinical history cannot be explained by findings on examination and investigation.

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“…When the patient first presented to the ED, it was presumed that the elevated creatinine was the result of anti-GBM disease and presumptive treatment was started immediately due to the potential for alveolar hemorrhage and rapid progression to renal failure [ 6 ]. It is thought that the development of anti-GBM disease may be preceded by the production of autoantibodies triggered by an unknown stimulus such as pulmonary irritation or kidney injury, and prompt intervention is warranted for the optimization of patient outcomes [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

“…Anti-GBM disease, previously known as Goodpasture syndrome, is a life-threatening form of vasculitis in which IgG autoantibodies bind to the alpha-3 chain of type IV collagen in alveolar and glomerular basement membranes [ 6 , 7 ]. It typically presents with hemoptysis resulting from alveolar hemorrhage in conjunction with rapidly progressive glomerulonephritis that can result in renal failure if untreated [ 6 ].…”

Section: Introductionmentioning

confidence: 99%

Complement-Mediated Thrombotic Microangiopathy in a Patient With Antiphospholipid Syndrome and Anti-glomerular Basement Membrane Antibodies

Gallant,

Zheng,

Hobbs

et al. 2023

Cureus

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Thrombotic microangiopathy (TMA) is a range of diseases characterized by thrombocytopenia, microangiopathic hemolytic anemia, and organ injury. Complement-mediated TMA is a rare, life-threatening subtype of TMA that occurs due to the uncontrolled activation of the alternative complement pathway in the absence of normal regulation, often resulting from deficiencies of various regulatory proteins. Anti-glomerular basement membrane (anti-GBM) disease, previously known as Goodpasture syndrome, is a life-threatening form of vasculitis in which immunoglobulin G autoantibodies bind to the alpha-3 chain of type IV collagen in alveolar and glomerular basement membranes. We present the case of a patient with a history of antiphospholipid syndrome who was diagnosed with complement-mediated TMA during hospital admission for elevated anti-GBM antibody titers discovered during an outpatient evaluation for elevated creatinine levels. Upon admission, treatment was started for presumed anti-GBM disease, including high-dose intravenous methylprednisolone injections and multiple plasmapheresis sessions. However, renal biopsy results showed no evidence of anti-GBM disease, but rather evidence of TMA. Subsequent laboratory studies revealed decreased complement levels, suggestive of a diagnosis of complement-mediated TMA. The patient was started on rituximab and eculizumab infusions, and she was discharged in stable condition after a 15-day hospitalization with outpatient appointments scheduled for genetic testing and further infusions. This case illustrates the importance of recognizing the key clinical and diagnostic features of complement-mediated TMA to promptly initiate appropriate therapy.

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An uncommon presentation of a rare disease: A case of anti-glomerular basement membrane disease without renal involvement

Cited by 3 publications

References 11 publications

Rituximab for the Treatment of Anti-glomerular Basement Membrane Disease with Isolated Diffuse Alveolar Hemorrhage

Rituximab for the Treatment of Anti-glomerular Basement Membrane Disease with Isolated Diffuse Alveolar Hemorrhage

Hemoptysis of Uncertain Cause Leading to Delayed Diagnosis in an Elderly Lady With Anti-glomerular Basement Membrane Disease

Complement-Mediated Thrombotic Microangiopathy in a Patient With Antiphospholipid Syndrome and Anti-glomerular Basement Membrane Antibodies

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