Anti-glomerular Basement Membrane Disease with Antiphospholipid Syndrome

Torigoe, Miki; Obata, Yoko; Kitamura, Mineaki; Hara, Shigeo; Fukuoka, Junya; Nishino, Tomoya

doi:10.2169/internalmedicine.4943-20

Cited by 2 publications

(3 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Several cases of anti-GBM disease with TMA have been reported [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20]; however, the clinical characteristics of this phenomenon remain unclear. Therefore, we conducted a literature review on this phenomenon (Table 2).…”

Section: Discussionmentioning

confidence: 99%

“…Autoimmune diseases, such as systemic lupus erythematosus, systemic sclerosis, antiphospholipid syndrome, and anti-neutrophil cytoplasmic autoantibody (ANCA)associated vasculitis, have been reported to be the cause of secondary TMA [2,3,5]. Though there are some existing case reports of anti-GBM disease with TMA [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20], the clinical features of this phenomenon have not been described extensively.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Clinical characteristics of anti-GBM disease with thrombotic microangiopathy: a case report and literature review

et al. 2023

View full text Add to dashboard Cite

The coexistence of anti-glomerular basement membrane (anti-GBM) disease with thrombotic microangiopathy (TMA) is rarely encountered, and the clinical characteristics of this phenomenon are not well known. A 76-year-old Japanese woman with a history of idiopathic pulmonary disease was diagnosed with anti-GBM disease due to rapidly progressive glomerulonephritis and a positive anti-GBM antibody test result. We treated the patient with hemodialysis, glucocorticoids, and plasmapheresis. During treatment, the patient suddenly became comatose. TMA was then diagnosed because of thrombocytopenia and microangiopathic hemolytic anemia. The activity of a disintegrin-like and metalloproteinase with thrombospondin type 1 motif 13 (ADAMTS-13) was retained at 48%. Although we continued the treatment, the patient died of respiratory failure. An autopsy revealed the cause of respiratory failure to be an acute exacerbation of interstitial pneumonia. The clinical findings of the renal specimen indicated anti-GBM disease; however, there were no lesions suggestive of TMA. A genetic test did not reveal an apparent genetic mutation of the atypical hemolytic uremic syndrome. We conducted a literature review of past case reports of anti-GBM disease with TMA. The following clinical characteristics were obtained. First, 75% of the cases were reported in Asia. Second, TMA tended to appear during the treatment course for anti-GBM disease and usually resolved within 12 weeks. Third, ADAMTS-13 activity was retained above 10% in 90% of the cases. Fourth, central nervous system manifestations occurred in more than half of the patients. Fifth, the renal outcome was very poor. Further studies are required to understand the pathophysiology of this phenomenon.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Clinical characteristics of anti-GBM disease with thrombotic microangiopathy: a case report and literature review

et al. 2023

View full text Add to dashboard Cite

show abstract

“…The possibility of anti-GBM disease was of particular concern in this patient due to her history of APS. To the best of the authors’ knowledge, only one case report exists discussing the concurrence of these two diseases, and that the patient’s clinical course was complicated by renal failure requiring hemodialysis, alveolar hemorrhage, convulsions, and a prolonged ICU stay [ 13 ]. Hence, presumptive treatment for anti-GBM disease was begun immediately upon the patient’s arrival at the hospital in the hopes of preventing progression to alveolar hemorrhage and hemodialysis.…”

Section: Discussionmentioning

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

View full text Add to dashboard Cite

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.

show abstract

Anti-glomerular Basement Membrane Disease with Antiphospholipid Syndrome

Cited by 2 publications

References 18 publications

Clinical characteristics of anti-GBM disease with thrombotic microangiopathy: a case report and literature review

Clinical characteristics of anti-GBM disease with thrombotic microangiopathy: a case report and literature review

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

Contact Info

Product

Resources

About