Case report: Thrombotic thrombocytopenic purpura in a pregnant woman with lupus membranous nephropathy: a diagnostic challenge
A 27-year-old female at 20th week of pregnancy was admitted with edema, foamy urine, but normal blood pressure. Her blood count was normal, she had proteinuria of 3 g/day, creatinine 0.4 mg/dl, albumin 2.4 g/dl, and cholesterol 355 mg/dl. Antinuclear antibodies 1/160, but Anti-DNA, anticardiolipin a...
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Frontiers Media S.A.
2024-02-01
|
| Series: | Frontiers in Nephrology |
| Subjects: | |
| Online Access: | https://www.frontiersin.org/articles/10.3389/fneph.2024.1343594/full |
| _version_ | 1797324012519424000 |
|---|---|
| author | Marina Leiva Gustavo Navarro J Daniel Carpio Leopoldo Ardiles |
| author_facet | Marina Leiva Gustavo Navarro J Daniel Carpio Leopoldo Ardiles |
| author_sort | Marina Leiva |
| collection | DOAJ |
| description | A 27-year-old female at 20th week of pregnancy was admitted with edema, foamy urine, but normal blood pressure. Her blood count was normal, she had proteinuria of 3 g/day, creatinine 0.4 mg/dl, albumin 2.4 g/dl, and cholesterol 355 mg/dl. Antinuclear antibodies 1/160, but Anti-DNA, anticardiolipin antibodies and lupus anticoagulant were negative, with normal serum C3 and C4. A renal biopsy showed secondary membranous glomerulopathy, most likely lupus class V pure. Steroids, azathioprine, and aspirin were initiated, up to 28 weeks of pregnancy, when she developed severe hypertension, photopsia, headache, anasarca, extensive bruising of the extremities, severe anemia, thrombocytopenia, and creatinine rose to 2.09 mg/dl with preserved diuresis. A female infant, 1045 grams, was delivered by emergency caesarean section. Following the surgery, she experienced diplopia, dysarthria, bradypsychia, and sensory alterations in the lower extremities, necessitating emergency hemodialysis due to pulmonary congestion. Blood smear revealed schistocytes, LDH elevated at 1148 IU/L, while transaminases and liver function remained normal, suggesting thrombotic thrombocytopenic purpura. ADAMTS13 revealed 6% activity with the presence of inhibitor. Mycophenolate and daily plasmapheresis with fresh frozen plasma replacement yielded unsatisfactory response, unaffected by the addition of methylprednisolone pulses and rituximab. Eventually, intravenous cyclophosphamide was introduced, resulting in complete hematological remission and normalization of ADAMTS13, however dialysis-dependence persisted and four years later, right renal cancer prompted bilateral nephrectomy. After a total follow-up of six years, she remained free of neoplastic recurrence and lupus activity, receiving prednisone and hydroxychloroquine. The differential diagnosis of microangiopathic syndrome in a pregnant lupus patient is discussed. |
| first_indexed | 2024-03-08T05:37:19Z |
| format | Article |
| id | doaj.art-dda4a27c6179463bbabd68da8808395b |
| institution | Directory Open Access Journal |
| issn | 2813-0626 |
| language | English |
| last_indexed | 2024-03-08T05:37:19Z |
| publishDate | 2024-02-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Nephrology |
| spelling | doaj.art-dda4a27c6179463bbabd68da8808395b2024-02-05T16:28:28ZengFrontiers Media S.A.Frontiers in Nephrology2813-06262024-02-01410.3389/fneph.2024.13435941343594Case report: Thrombotic thrombocytopenic purpura in a pregnant woman with lupus membranous nephropathy: a diagnostic challengeMarina Leiva0Gustavo Navarro1J Daniel Carpio2Leopoldo Ardiles3Department of Nephrology, Hospital Intercultural de Nueva Imperial, Nueva Imperial, ChileLaboratory of Nephrology, Institute of Medicine, Faculty of Medicine, Universidad Austral de Chile, Valdivia, ChileInstitute of Anatomy, Histology and Pathology, Faculty of Medicine, Universidad Austral de Chile, Valdivia, ChileLaboratory of Nephrology, Institute of Medicine, Faculty of Medicine, Universidad Austral de Chile, Valdivia, ChileA 27-year-old female at 20th week of pregnancy was admitted with edema, foamy urine, but normal blood pressure. Her blood count was normal, she had proteinuria of 3 g/day, creatinine 0.4 mg/dl, albumin 2.4 g/dl, and cholesterol 355 mg/dl. Antinuclear antibodies 1/160, but Anti-DNA, anticardiolipin antibodies and lupus anticoagulant were negative, with normal serum C3 and C4. A renal biopsy showed secondary membranous glomerulopathy, most likely lupus class V pure. Steroids, azathioprine, and aspirin were initiated, up to 28 weeks of pregnancy, when she developed severe hypertension, photopsia, headache, anasarca, extensive bruising of the extremities, severe anemia, thrombocytopenia, and creatinine rose to 2.09 mg/dl with preserved diuresis. A female infant, 1045 grams, was delivered by emergency caesarean section. Following the surgery, she experienced diplopia, dysarthria, bradypsychia, and sensory alterations in the lower extremities, necessitating emergency hemodialysis due to pulmonary congestion. Blood smear revealed schistocytes, LDH elevated at 1148 IU/L, while transaminases and liver function remained normal, suggesting thrombotic thrombocytopenic purpura. ADAMTS13 revealed 6% activity with the presence of inhibitor. Mycophenolate and daily plasmapheresis with fresh frozen plasma replacement yielded unsatisfactory response, unaffected by the addition of methylprednisolone pulses and rituximab. Eventually, intravenous cyclophosphamide was introduced, resulting in complete hematological remission and normalization of ADAMTS13, however dialysis-dependence persisted and four years later, right renal cancer prompted bilateral nephrectomy. After a total follow-up of six years, she remained free of neoplastic recurrence and lupus activity, receiving prednisone and hydroxychloroquine. The differential diagnosis of microangiopathic syndrome in a pregnant lupus patient is discussed.https://www.frontiersin.org/articles/10.3389/fneph.2024.1343594/fullthrombocytopeniamicroangiopathic anemialupus (SLE)acute kidney injuryADAMST 13 |
| spellingShingle | Marina Leiva Gustavo Navarro J Daniel Carpio Leopoldo Ardiles Case report: Thrombotic thrombocytopenic purpura in a pregnant woman with lupus membranous nephropathy: a diagnostic challenge Frontiers in Nephrology thrombocytopenia microangiopathic anemia lupus (SLE) acute kidney injury ADAMST 13 |
| title | Case report: Thrombotic thrombocytopenic purpura in a pregnant woman with lupus membranous nephropathy: a diagnostic challenge |
| title_full | Case report: Thrombotic thrombocytopenic purpura in a pregnant woman with lupus membranous nephropathy: a diagnostic challenge |
| title_fullStr | Case report: Thrombotic thrombocytopenic purpura in a pregnant woman with lupus membranous nephropathy: a diagnostic challenge |
| title_full_unstemmed | Case report: Thrombotic thrombocytopenic purpura in a pregnant woman with lupus membranous nephropathy: a diagnostic challenge |
| title_short | Case report: Thrombotic thrombocytopenic purpura in a pregnant woman with lupus membranous nephropathy: a diagnostic challenge |
| title_sort | case report thrombotic thrombocytopenic purpura in a pregnant woman with lupus membranous nephropathy a diagnostic challenge |
| topic | thrombocytopenia microangiopathic anemia lupus (SLE) acute kidney injury ADAMST 13 |
| url | https://www.frontiersin.org/articles/10.3389/fneph.2024.1343594/full |
| work_keys_str_mv | AT marinaleiva casereportthromboticthrombocytopenicpurpurainapregnantwomanwithlupusmembranousnephropathyadiagnosticchallenge AT gustavonavarro casereportthromboticthrombocytopenicpurpurainapregnantwomanwithlupusmembranousnephropathyadiagnosticchallenge AT jdanielcarpio casereportthromboticthrombocytopenicpurpurainapregnantwomanwithlupusmembranousnephropathyadiagnosticchallenge AT leopoldoardiles casereportthromboticthrombocytopenicpurpurainapregnantwomanwithlupusmembranousnephropathyadiagnosticchallenge |