Thrombotic thrombocytopenic purpura complicating Sjögren’s syndrome with crescentic glomerulonephritis and membranous nephritis

Abe, Haruo; Tsuboi, Norioki; Yukawa, Sonosuke; Tsuji, Souichirou; Hayashi, Haeru; Yukawa, Naoichiro; Takanashi, Hirofumi; Tahara, Koichiro; Tonozuka, Norihiko; Hayashi, Tohru

doi:10.3109/s10165-004-0287-4

Cited by 7 publications

(5 citation statements)

References 16 publications

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“…In addition, 19 case reports including 22 patients with SS and MN were also obtained (Table 4). 24‐42 The medium age of these patients was 55 years and 76.2% were female. Sixteen cases had other concurrent diseases, with 3 lymphomas and 13 autoimmune disorders such as rheumatoid arthritis, thyroiditis, Graves’ disease, primary biliary cirrhosis and so on.…”

Section: Resultsmentioning

confidence: 96%

Sjögren’s syndrome complicated with membranous nephropathy, a cause or coincidence?

et al. 2021

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Background Sjögren's syndrome (SS) has been a well‐documented cause of secondary membranous nephropathy (MN); however, the prevalence is quite low. Since primary MN is also a common disease in middle age, whether MN is secondary to SS or just coincidence remains uncertain. The detection of phospholipase A2 receptor (PLA2R), which is most often positive in idiopathic MN, has been rarely reported in such cases. Methods We retrospectively studied 13 cases diagnosed with MN and SS in Huashan Hospital between 2009 and 2020, and performed PLA2R detection. We also review the literature by searching in the PubMed database. Results Among the 13 patients, 8 were found to be PLA2R‐positive and 5 were negative. Nine patients were female. All but 1 patients had normal renal function at the time of biopsy. All patients showed positive anti‐nuclear antibody and anti‐SSA. Two of the 8 PLA2R‐positive patients showed positive anti‐SSB and 1/8 had mild hypocomplementemia, while all 5 PLA2R‐negative patients had positive anti‐SSB and 3 showed hypocomplementemia. Renal biopsy revealed focal MN and markedly mesangial hyperplasia in PLA2R‐negative patients. Mesangial electron deposits were observed in 1 PLA2R‐positive patient in small amounts, and in 3 PLA2R‐negative patients with 2 in large amounts. During follow‐up, 2 patients in the PLA2R‐negative group presented with progressively decreasing serum complement levels, and another one was diagnosed with systemic lupus erythematosus (SLE). Conclusions In patients with both MN and SS, PLA2R‐negative MN should be considered as a secondary form. Careful screen for SLE is necessary in these patients during follow‐up.

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Section: Resultsmentioning

confidence: 96%

Sjögren’s syndrome complicated with membranous nephropathy, a cause or coincidence?

et al. 2021

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“…In the present case, the acquired TTP developed during the conservative treatment of idiopathic MN. Secondary MN and secondary TMA can be caused by collagen diseases such as systemic lupus erythematosus and Sjogren's syndrome, malignancies, and certain drugs, and can sometimes coexist [4]. In contrast, idiopathic MN and acquired TTP have different etiologies, and their coexistence is unusual.…”

Section: Discussionmentioning

confidence: 99%

Thrombotic thrombocytopenic purpura developed during the conservative treatment of anti-phospholipase A2 receptor antibody-positive idiopathic membranous nephropathy: a case report

et al. 2020

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Background Idiopathic membranous nephropathy (MN) is one of the major glomerulonephritis that cause nephrotic syndrome. The phospholipase A2 receptor (PLA2R) has recently been identified as an endogenous antigen of idiopathic MN. Thrombotic thrombocytopenic purpura (TTP) is a disorder characterized by schistocytes, hemolytic anemia, thrombocytopenia, and organ dysfunction which occurs as a result of thrombi. Patients with acquired TTP have autoantibodies against a disintegrin and metalloprotease with thrombospondin type 1 motif 13 (ADAMTS13). These autoantibodies act as an inhibitor and cause ADAMTS13 deficiency. Idiopathic MN and acquired TTP are usually considered as independent autoimmune diseases. We experienced a patient who developed TTP during the conservative treatment of idiopathic MN, with the coexistence of ADAMTS13 inhibitor and anti-PLA2R antibody. Case presentation A 73-year-old man presented with thrombocytopenia, hemolytic anemia, disturbance of consciousness, and acute kidney injury after 4-year course of biopsy-proven idiopathic MN. ADAMTS13 activity was undetectable and the ADAMTS13 inhibitor was identified. Additionally, he was positive for anti-PLA2R antibody. The patient did not have any diseases that could cause secondary thrombotic microangiopathy, and he was diagnosed with acquired TTP. Steroid therapy and plasma exchange were initiated and the acquired TTP resolved. MN achieved remission 3 months after the anti-PLA2R antibody disappeared. Conclusions This is the first reported case of acquired TTP developed during conservative treatment of idiopathic MN, with both ADAMTS13 inhibitor and anti-PLA2R antibody positive at the onset of the TTP. The present case suggests that idiopathic MN might be associated with the development of some cases of acquired TTP.

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“…This is apparently a very rare association. A review of the literature reveals only eight additional cases of TTP or haemolytic uraemic syndrome (HUS) associated with pSS in about 40 years [22][23][24][25][26][27][28][29] (Table 1). These cases are similar in many respects, but they show variability in the extent of haemolysis (haemoglobin 6.8-11.0 g/dl), severity of thrombocytopaenia (platelets 5-104 9 10 3 /ll) and renal damage.…”

Section: Discussionmentioning

confidence: 99%

Thrombotic thrombocytopenic purpura with an autoantibody to ADAMTS13 complicating Sjögren’s syndrome: two cases and a literature review

Yamashita¹,

Takahashi²,

Kaneko³

et al. 2013

Modern Rheumatology

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An association between thrombotic thrombocytopenic purpura (TTP) and Sjögren's syndrome (SS) is rare. This is the first report of two patients with TTP who had inhibitory autoantibodies to ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type 1 repeats) complicating primary SS. A rapid diagnosis of TTP, which is a potentially lethal condition, made it possible to treat the two cases successfully. Only eight similar cases with TTP complicating SS have been reported in the literature. The possible presentation of primary SS without classic sicca symptoms, but with haematological abnormalities including TTP, should be recognised. Furthermore, it is important to measure ADAMTS13 activity and anti-ADAMTS13 antibodies, because TTP with SS seems to be a concurrent overlapping autoimmune disorder. We suggest that plasma exchanges in combination with corticosteroids should be administered as early as possible, since they appeared to be effective in treating TTP with SS, including in our cases.

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Thrombotic thrombocytopenic purpura complicating Sjögren’s syndrome with crescentic glomerulonephritis and membranous nephritis

Cited by 7 publications

References 16 publications

Sjögren’s syndrome complicated with membranous nephropathy, a cause or coincidence?

Sjögren’s syndrome complicated with membranous nephropathy, a cause or coincidence?

Thrombotic thrombocytopenic purpura developed during the conservative treatment of anti-phospholipase A2 receptor antibody-positive idiopathic membranous nephropathy: a case report

Thrombotic thrombocytopenic purpura with an autoantibody to ADAMTS13 complicating Sjögren’s syndrome: two cases and a literature review

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