Systemic lupus erythematosus and antineutrophilic cytoplasmic antibody-associated vasculitis overlap syndrome complicated by subarachnoid hemorrhage: case-based review

Khorolsky, Ciril; Castellano, Andrew; Comstock, David; Brinster, Nooshin; See, Sein Y; Garner, Bruce

doi:10.1007/s00296-018-4169-z

Cited by 7 publications

(7 citation statements)

References 22 publications

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“…SLE and AAV can be distinguished according to their own antibody spectrum, histopathological characteristics, and demographic characteristics. [ 12 ] However, some patients showed mixed clinical patterns, which met the diagnostic criteria of SLE and AAV. Jarrot et al [ 11 ] found that SLE associated with AAV may be a group of diseases called SLE/AAV overlapping syndrome.…”

Section: Discussionmentioning

confidence: 99%

Systemic lupus erythematosus and antineutrocytic cytoplasmic antibody-associated vasculitis overlap syndrome presenting mainly with alveolar hemorrhage: A case report and literature review

Yang,

Zhou

2023

Medicine

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Rationale: Systemic lupus erythematosus (SLE) and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are 2 different diseases that can manifest in the same person, which are known as SLE/AAV overlap syndrome. This overlap syndrome is difficult to diagnose, a high rate of missed diagnosis and misdiagnosis, and a poor prognosis. Patient concerns: A 52-year-old woman was diagnosed with SLE in 2019. She was readmitted to our hospital in October 2021 because of abdominal pain and melasma for 10 days. Diagnoses: She had positive anti-dsDNA, decreased complement C3 and C4, fever, polyarthralgia, and hemolytic anemia. She was diagnosed as microscopic polyangiitis according to the American College of Rheumatology 2022 AAV classification criteria (she had 4 items: no nasal lesions, eosinophils < 1 × 109, negative c/PR3-ANCA antibodies, and positive p-ANCA antibodies. The score was 6 points). Interventions: The patient was treated with methylprednisolone 200 mg and cyclophosphamide 0.2 g immunosuppressive therapy. Outcomes: After 2 months of follow-up, the patient’s symptoms, including abdominal pain, melena, hematuria, and hemoptysis, resolved completely. And she underwent a reexamination of chest computed tomography and the results showed the previous exudation had been absorbed. Lessons: AAV should be considered in lupus patients with the above symptoms, especially the progressive decrease of hemoglobin. Relevant examinations are needed to confirm the diagnosis. Early diagnosis and accurate treatment of SLE/AAV overlap syndrome are beneficial to patients’ better prognosis and control the treatment cost.

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Section: Discussionmentioning

confidence: 99%

Systemic lupus erythematosus and antineutrocytic cytoplasmic antibody-associated vasculitis overlap syndrome presenting mainly with alveolar hemorrhage: A case report and literature review

Yang,

Zhou

2023

Medicine

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“…Antineutrophil cytoplasmic autoantibodies (ANCA) are a group of autoimmune antibodies that are specific for the diagnosis of ANCA-associated vasculitis (AAV), which is an autoimmune disorder affecting small-sized, to a lesser degree, medium-sized vessels and renal injury is common in AAV which characterized by crescent formation with pauci-immune deposits [ 6 , 7 ]. ANCA are also found in the occasions of tumors [ 8 ], infections [ 9 ], other autoimmune diseases, such as systemic lupus erythematosus [ 10 ], or induction by certain medicine [ 11 ]. But AAV caused by brucellosis was rarely reported.…”

Section: Introductionmentioning

confidence: 99%

Coexistence of cryoglobulinemia and ANCA-associated vasculitis in a chronic brucellosis patient -a case report and literature review

Jiao

Liu

et al. 2023

BMC Infect Dis

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Background The renal involvement of brucellosis is not common. Here we reported a rare case of chronic brucellosis accompanied by nephritic syndrome, acute kidney injury, the coexistence of cryoglobulinemia and antineutrophil cytoplasmic autoantibodies (ANCA) associated vasculitis (AAV) superimposed on iliac aortic stent implantation. The diagnosis and treatment of the case are instructive. Case presentation A 49-year-old man with hypertension and iliac aortic stent implantation was admitted for unexplained renal failure with signs of nephritic syndrome, congestive heart failure, moderate anemia and livedoid change in the left sole with pain. His past history included chronic brucellosis and he just underwent the recurrence and completed the 6 weeks of antibiotics treatment. He demonstrated positive cytoplasmic/proteinase 3 ANCA, mixed type cryoglobulinemia and decreased C3. The kidney biopsy revealed endocapillary proliferative glomerulonephritis with a small amount of crescent formation. Immunofluorescence staining revealed only C3-positive staining. In accordance with clinical and laboratory findings, post-infective acute glomerulonephritis superimposed with AAV was diagnosed. The patient was treated with corticosteroids and antibiotics and sustained alleviation of renal function and brucellosis was achieved during the course of a 3-month follow-up. Conclusions Here we describe the diagnostic and treatment challenge in a patient with chronic brucellosis related glomerulonephritis accompanied by the coexistence of AAV and cryoglobulinemia. Renal biopsy confirmed the diagnosis of postinfectious acute glomerulonephritis overlapping with ANCA related crescentic glomerulonephritis, which was not ever reported in the literature. The patient showed a good response to steroid treatment which indicated the immunity-induced kidney injury. Meanwhile, it is essential to recognize and actively treat the coexisting brucellosis even when there are no clinical signs of the active stage of infection. This is the critical point for a salutary patient outcome for brucellosis associated renal complications.

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“…4 SLE/AAV overlap syndrome is an inflammatory disease characterized by clinical features of both SLE and AAV. 5 According to recent literature, any organ may be affected in the SLE/AAV overlap syndrome. It is to be noted that kidney is most commonly involved, with rapidly progressive glomerulonephritis as the most common clinical manifestation.…”

Section: Introductionmentioning

confidence: 99%

“…It is to be noted that kidney is most commonly involved, with rapidly progressive glomerulonephritis as the most common clinical manifestation. 5,6 Other systemic involvement of the SLE/AAV overlap syndrome include cerebral ischemia, central nervous system vasculitis, subarachnoid hemorrhage, lupus granulomatous pneumonitis, pulmonary hemorrhage, arthritis, serositis, cytopenia, rhinosinusitis, and thrombopenia. [5][6][7] As we have known so far, few previous cases have reported nasal and palatal perforation in this disorder.…”

Section: Introductionmentioning

confidence: 99%

Coincidence of Systemic Lupus Erythematosus and ANCA-Associated Vasculitis: A Case Report with Perforation of Nasal Septum and Palate

Liu,

Yu,

et al. 2023

JIR

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Background An overlap of systemic lupus erythematosus (SLE) and antineutrophil cytoplasmic antibodies- (ANCA-) associated vasculitis (AAV) is extremely uncommon and no clear definition has been proposed. The SLE/AAV overlap syndrome mainly affects kidney, blood count, nervous system and lung. However, few previous cases reported nasal septal and palatal perforation in this disorder. Case Presentation We presented a case of a 16-year-old female with a 6-month history of SLE, developed perforation of the nasal septum and palate. She was diagnosed with SLE due to facial malar rash, oral ulcer, increased erythrocyte sedimentation rate (ESR), low complement levels, and positive anti-Smith antibody. Approximately 6 months later, she had a perforation of the nasal septum and palate with positive anti-proteinase 3 antibody (anti-PR3-ANCA). A nasal endoscopic biopsy revealed an inflammatory polyp with chronic suppurative inflammation and inflammatory granulomatous hyperplasia. In this case, the clinical, biological, radiological, and histological findings substantiated the diagnosis of AAV. Infections, drug abuse, malignancies, IgG4-related disease (IgG4-RD) and trauma were excluded. So we diagnosed her with SLE/AAV overlap syndrome. Conclusion When a patient’s symptoms cannot be explained by one disease, we need to consider the overlapping of two diseases, especially in patients with autoimmune diseases.

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Systemic lupus erythematosus and antineutrophilic cytoplasmic antibody-associated vasculitis overlap syndrome complicated by subarachnoid hemorrhage: case-based review

Cited by 7 publications

References 22 publications

Systemic lupus erythematosus and antineutrocytic cytoplasmic antibody-associated vasculitis overlap syndrome presenting mainly with alveolar hemorrhage: A case report and literature review

Systemic lupus erythematosus and antineutrocytic cytoplasmic antibody-associated vasculitis overlap syndrome presenting mainly with alveolar hemorrhage: A case report and literature review

Coexistence of cryoglobulinemia and ANCA-associated vasculitis in a chronic brucellosis patient -a case report and literature review

Coincidence of Systemic Lupus Erythematosus and ANCA-Associated Vasculitis: A Case Report with Perforation of Nasal Septum and Palate

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