Sustained Remission of Antineutrophil Cytoplasmic Antibody-Mediated Glomerulonephritis and Nephrotic Syndrome in Mixed Connective Tissue Disease

Konstantinov, Konstantin N.; Harris, Angela P.; Barry, Marc; Murata, Glen H.; Tzamaloukas, Antonios H.

doi:10.4021/jocmr1391w

Cited by 6 publications

(3 citation statements)

References 15 publications

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“…Comparable to other autoimmune diseases treatment strategies consist in immunosuppressive drugs like corticosteroids alone or together with azathioprine and/or cyclophosphamide or biologics [2,10]. In acute stages of these diseases the drugs can be applied in high doses as pulse therapy.…”

Section: Introductionmentioning

confidence: 99%

Therapeutic apheresis in immunocomplex nephritis: A case report of a successful treatment.

Bambauer¹,

Schiel²

2018

clinical-nephrology

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The advantages of the Therapeutic Apheresis (TA) with membrane modules, which became available since the mid-1970s, are a complete separation of the blood cells from the plasma. Autoimmune diseases have an immune pathogenesis and can produce auto antibodies (ab) and Circulating Immune Complexes (CIC), which are the origin of inflammation in various organs. The most patients with these diseases have a poor prognosis without treatment. TA in combination with immunosuppressive drugs has brought an increase in the survival rates over the last 40 years. The origin of autoimmune diseases is still generally unknown. The variety of autoimmune diseases ranges from those diseases in which autoimmunization is solely responsible for the condition (e.g., autoimmune hemolytic anemia) to those in which it possibly has a major influence on the course of the disease (e.g., rheumatoid arthritis), and those in which the autoimmunization results are probably only of diagnostic importance. The auto antibodies (auto-abs) activate different immunological processes which are selfdestructive for the organs. These auto antibodies can be directed to all blood cells, too. With a case report of a woman, who was treated with Therapeutic Plasma Exchange (TPE) intermittently since her childhood, the authors show the efficacy of TPE in autoimmune diseases. The patients have had immunocomplex nephritis with a severe nephrotic syndrome which was diagnosed by a renal biopsy in the age of 14 months of the child. In the now more than 30 years old woman acute attacks of immunocomplex nephritis with nephrotic syndrome could be well controlled by TPE without excessive increases in the dosage of immunosuppressive drugs and a significant impairment in renal function.

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Section: Introductionmentioning

confidence: 99%

Therapeutic apheresis in immunocomplex nephritis: A case report of a successful treatment.

Bambauer¹,

Schiel²

2018

clinical-nephrology

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“…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%

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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“…The need for immunosuppressive therapy in renal disease has mostly been reported in MCTD and accompanying histopathological features, such as ANCA-mediated glomerulonephritis, or SLE-associated glomerulonephritis [6,7].…”

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confidence: 99%

Mixed connective tissue disease and severe renal disease – an apparent misconception?

Pinheiro¹,

Madureira²,

Rato³

et al. 2022

Reumatologia

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Sustained Remission of Antineutrophil Cytoplasmic Antibody-Mediated Glomerulonephritis and Nephrotic Syndrome in Mixed Connective Tissue Disease

Cited by 6 publications

References 15 publications

Therapeutic apheresis in immunocomplex nephritis: A case report of a successful treatment.

Therapeutic apheresis in immunocomplex nephritis: A case report of a successful treatment.

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

Mixed connective tissue disease and severe renal disease – an apparent misconception?

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