Mixed Connective Tissue Disease Associated with MPO-ANCA-positive Polyangiitis

Kitaura, Keisuke; Miyagawa, Tomoko; Asano, Kenichiro; Oouchi, Sachiko; Miki, Takashi; Fujisawa, Takashi; Ishida, Kenjiro

doi:10.2169/internalmedicine.45.1703

Cited by 22 publications

(13 citation statements)

References 10 publications

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“…In conclusion, we report overlapping MPO-ANCA-related polyangiitis and renal involvement of MCTD in this patient. In the previous reports of MCTD patients with MPO-ANCA-related necrotizing glomerulonephritis, immunofluorescence analysis revealed pauci-immune necrotizing glomerulonephritis (6)(7)(8), which implies that patients without the renal involvement of MCTD developed MPO-ANCA-related necrotizing glomerulonephritis. This might be the first report on the simultaneous occurrence of the immunecomplex glomerulonephritis of MCTD and MPO-ANCArelated crescentic glomerulonephritis.…”

Section: Discussionmentioning

confidence: 81%

“…Furthermore, acute loss of renal function caused by myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA)-related necrotizing glomerulonephritis has been rarely reported in MCTD patients (6)(7)(8). Therefore, lupus nephritis or MPO-ANCA-related necrotizing glomerulonephritis should be considered to be a possible involvement in MCTD patients with rapidly progressive glomerulonephritis (RPGN).…”

Section: Introductionmentioning

confidence: 99%

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Mixed Connective Tissue Disease Developing into MPO-ANCA-positive Polyangiitis

et al. 2011

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Renal involvement of mixed connective tissue disease (MCTD) shows systemic lupus erythematosus (SLE)-like immune complex glomerulonephritis. The prognosis of this condition is generally good. We report the case of an elderly female patient with MCTD who developed autoimmune pleurisy and rapidly progressive glomerulonephritis. Myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) was positive with a titer of 59.0 EU. Anti-DNA antibody and complement levels were normal. Renal biopsy revealed crescentic glomerulonephritis and mild mesangial proliferation. However, immunofluorescence examination revealed immune-complex glomerulonephritis. These findings suggest that the renal involvement of MCTD developed concurrently with MPO-ANCA-related glomerulonephritis.

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Section: Discussionmentioning

confidence: 81%

Section: Introductionmentioning

confidence: 99%

Mixed Connective Tissue Disease Developing into MPO-ANCA-positive Polyangiitis

et al. 2011

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“…The most frequent clinical manifestations were glomerulonephritis and interstitial pneumonia (80% each, respectively). Of note, three of 10 patients (30%) suffered from alveolar hemorrhage, and two of three patients with alveolar hemorrhage died in spite of intensive treatment (Inada et al 1999;Kitaura et al 2006). With regard to therapy, all patients received corticosteroids and eight received methylprednisolone pulse therapy.…”

Section: Discussionmentioning

confidence: 99%

“…Also, progressive renal disease (rapidly progressive glomerulonephritis, RPGN) seems to be associated with a severe outcome in such patients. Actually, of all patients with a fatal outcome associated with glomerulonephritis, two of three patients had RPGN (Inada et al 1999;Kitaura et al 2006). All patients received corticosteroids, and the major immunosuppressive agent used was cyclophosphamide (60%).…”

Section: Discussionmentioning

confidence: 99%

“…Complication of MPA in patients with MCTD is rare. So far, only nine MCTD cases accompanied by MPO-ANCApositive MPA have been described (Inada et al 1999;Makita et al 2000;Hernández-Molina et al 2006;Kitaura et al 2006;Hong et al 2010;Murakami et al 2011;Konstantinov et al 2013;Murakami et al 2013;Sun et al 2014). Here we report a rare case of MCTD complicated by MPA with alveolar hemorrhage, responding well to immunosuppressive treatment.…”

Section: Introductionmentioning

confidence: 99%

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Successful Immunosuppressive Treatment of Mixed Connective Tissue Disease Complicated by Microscopic Polyangiitis

Sato

Yashiro

Matsuoka

et al. 2016

Tohoku J. Exp. Med.

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Mixed connective tissue disease (MCTD) is characterized by a combination of clinical features of systemic lupus erythematosus, systemic sclerosis, and polymyositis with elevated antibodies to U1 small nuclear ribonucleoprotein (U1-RNP). MCTD is often accompanied by interstitial lung disease as pulmonary involvement. On the other hand, microscopic polyangiitis (MPA) is a systemic autoimmune disease characterized by the inflammation of small vessels (arterioles, capillaries, and venules) mainly affecting the lung and kidney. MPA is associated with elevated serum anti-neutrophil cytoplasmic antibody (ANCA). Complication of MPA in patients with MCTD is rare. So far, only nine case reports of MCTD complicated by MPA with serum myeloperoxidase-specific ANCA (MPO-ANCA) are available. Here, we describe a 64-year-old male suffering from MCTD with MPA. The patient developed interstitial pneumonia with alveolar hemorrhage accompanied by myositis, scleroderma, and elevated anti-U1-RNP antibody and MPO-ANCA levels with substantial systemic inflammation. Strong immunosuppressive therapy (corticosteroid, intravenous immunoglobulin, and cyclosporine A) ameliorated the myositis, interstitial lung disease, and inflammation, with the decrease of MPO-ANCA levels, despite that severe lung complications are often associated with poor outcomes. In conclusion, MCTD may be accompanied by MPA with alveolar hemorrhage. Severe lung complications may indicate a poor outcome, and therefore prompt immunosuppressive treatment should be performed in such patients.

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Guidelines on the use of therapeutic apheresis in clinical practice—Evidence‐based approach from the apheresis applications committee of the American Society for Apheresis

Szczepiórkowski

Winters

Bandarenko

et al. 2010

J of Clinical Apheresis

498

285

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The American Society for Apheresis (ASFA) Apheresis Applications Committee is charged with a review and categorization of indications for therapeutic apheresis. Beginning with the 2007 ASFA Special Issue (fourth edition), the subcommittee has incorporated systematic review and evidence-based approach in the grading and categorization of indications. This Fifth ASFA Special Issue has further improved the process of using evidence-based medicine in the recommendations by refining the category definitions and by adding a grade of recommendation based on widely accepted GRADE system. The concept of a fact sheet was introduced in the Fourth edition and is only slightly modified in this current edition. The fact sheet succinctly summarizes the evidence for the use of therapeutic apheresis. The article consists of 59 fact sheets devoted to each disease entity currently categorized by the ASFA as category I through III. Category IV indications are also listed.

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Mixed Connective Tissue Disease Associated with MPO-ANCA-positive Polyangiitis

Abstract: The patient was a 42-year-old woman diagnosed as having MCTD and Sjögren's syndrome in 1989, and who

Cited by 22 publications

References 10 publications

Mixed Connective Tissue Disease Developing into MPO-ANCA-positive Polyangiitis

Mixed Connective Tissue Disease Developing into MPO-ANCA-positive Polyangiitis

Successful Immunosuppressive Treatment of Mixed Connective Tissue Disease Complicated by Microscopic Polyangiitis

Guidelines on the use of therapeutic apheresis in clinical practice—Evidence‐based approach from the apheresis applications committee of the American Society for Apheresis

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