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Vasculitides
Published in Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang, Roxburgh's Common Skin Diseases, 2022
Ivy M. Obonyo, Virginia A. Jones, Kayla A. Clark, Maria M. Tsoukas
Management: Cryoglobulinemic vasculitis is most often due to an underlying lymphoproliferative (type I), infectious (type II/III) or autoimmune cause (type II/III). When lymphoproliferative, treatment can include plasmapheresis, rituximab, or corticosteroids. When infectious in nature, usually due to Hepatitis C Virus (HCV), treatment includes antiviral therapies (PegIFN/ribivarin) when mild, and rituximab ± plasmapheresis prior to antiviral therapeutics when severe. When due to an underlying autoimmune disease, such as Sjogren’s, treatment includes immunosuppressives, such as high-dose corticosteroids, rituximab, cyclophosphamide, and mycophenolate mofetil, or plasmapheresis.
Sjögren Syndrome
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Complications associated with primary Sjögren syndrome may be treated with standard protocols: (i) parotid swelling (short-term oral corticosteroids; antibiotic treatment); (ii) arthritis (hydroxychloroquine; nonsteroidal anti-inflammatory drug [NSAIDs]; short-term oral/intraarticular corticosteroids; other disease-modifying antirheumatic drug [DMARDs] as with rheumatoid arthritis); (iii) interstitial lung disease (corticosteroids, oral or intravenous; cyclophosphamide for active alveolitis; pirfenidone, nintedanib); (iv) tubulointerstitial nephritis (potassium and bicarbonate replacement); (v) glomerulonephritis (corticosteroids, oral or intravenous; cyclophosphamide; mycofenolate mofetil); (vi) peripheral neuropathy (gabapentinoids; corticosteroids; intravenous immunoglobulins [IVIg]); (vi) cryoglobulinemic vasculitis (corticosteroids, plasmapheresis) [36–38].
Current perspectives on the diagnosis, assessment, and management of vasculitic neuropathy
Published in Expert Review of Neurotherapeutics, 2022
Yuki Fukami, Haruki Koike, Masahisa Katsuno
Evidence for the use of immunosuppressive and immunomodulatory therapies against the neuropathy of Sjögren’s syndrome is rare as there are no available RCTs. Management of these symptoms depends on the presence of cryoglobulinemic vasculitis, the severity and course of the disease, and the type of neuropathy. Patients diagnosed with vasculitis on muscle biopsy have better neurological outcomes than those without [95]. Interestingly, the presence of necrotizing vasculitis is the only predictor of better neurological outcomes [96]. Vasculitis, especially cryoglobulinemic vasculitis, usually requires prompt immunosuppressive therapy such as rituximab. The benefit of steroids and immunosuppressive agents is limited, and rituximab appears less effective in the absence of cryoglobulinemia [97]. However, IVIg therapy appears to be effective in a small number of steroid-resistant patients [98].
Hepatitis B virus infection as a risk factor for chronic kidney disease
Published in Expert Review of Clinical Pharmacology, 2019
Fabrizio Fabrizi, Roberta Cerutti, Ezequiel Ridruejo
Clinical practice guidelines for the treatment of HBV-associated cryoglobulinemic vasculitis have not been issued to date. We suggest to start antiviral therapy with NAs (or peg-IFN) for patients with HBV-related cryoglobulinemic vasculitis whose disease severity is mild to moderate. For those having severe disease (i.e. progressive motor neuropathy, rapid kidney failure, skin ulcers), we suggest plasma-exchange, rituximab, and/or conventional immunosuppressive agents. Concomitant therapy with NAs (or peg-IFN) is recommended as the risk of HBV reactivation has been noted. The use of immunosuppressive agents should be very selective due to its numerous side-effects and risk of HBV reactivation. Dose of NAs (or peg-IFN) should be tailored according to the levels of kidney function. The most important NAs are currently entecavir and tenofovir-pharmacokinetic studies have shown a better kidney safety profile with tenofovir alafenamide compared with tenofovir disoproxil fumarate [45].
Cryoglobulinemic vasculitis with primary Sjögren’s syndrome: A case report
Published in Modern Rheumatology, 2018
Jumpei Hasegawa, Noriko Hayami, Junichi Hoshino, Tatsuya Suwabe, Keiichi Sumida, Koki Mise, Toshiharu Ueno, Masayuki Yamanouchi, Naoki Sawa, Kenichi Ohashi, Takeshi Fujii, Kenmei Takaichi, Yoshifumi Ubara
Cryoglobulinemic vasculitis is a disorder caused by cryoglobulins, which are immunoglobulins that precipitate in vivo at temperatures lower than 37 °C and cause multiorgan damage [1]. Mixed (type II) cryoglobulinemia is caused by immune complexes between monoclonal immunoglobulin (Ig) M and IgG, and it presents with vasculitis involving small- to medium-sized vessels in the skin, joints, nerves, and/or kidneys. The majority of patients have an underlying infection with hepatitis C virus (HCV), but autoimmune diseases such as Sjögren’s syndrome can also present with mixed cryoglobulinemia [2]. Although it has been reported that rituximab is effective for cryoglobulinemic vasculitis in patients with HCV infection [3,4], there is little information about the management of cryoglobulinemic vasculitis associated with Sjögren’s syndrome. Accordingly, we report the clinical course and response to treatment in a patient with Sjögren’s syndrome and cryoglobulinemic vasculitis.