Evidence for immunoglobulin-mediated vasculitis caused by monoclonal gammopathy in monoclonal gammopathy of unclear significance prompting oncologic treatment

Drerup, Christian; Metze, Dieter; Ehrchen, Jan; Mitschang, Carolin; Neufeld, Matthias; Sunderkötter, Cord

doi:10.1016/j.jdcr.2019.01.013

Cited by 12 publications

(12 citation statements)

References 11 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Umemura et al 14 describe a case of IgA-associated leukocytoclastic vasculitis associated with MGUS of an IgA lambda chain. Drerup et al 15 present two similar cases with typical signs of immune-mediated small vessel vasculitis and demonstrate paraproteinaemia can cause small vessel vasculitis. Furthermore, these case reports also demonstrated a good response to immunosuppressive treatment with a reduction in the paraprotein and vasculitis manifestations.…”

Section: Discussionmentioning

confidence: 95%

See 1 more Smart Citation

Monoclonal gammopathy of undetermined significance causing large vessel vasculitis

2022

View full text Add to dashboard Cite

A man in his late 50s presented with unilateral pain and discolouration of his fourth and fifth toes suggestive of digital ischaemia. He had a medical history of two unprovoked venous thromboembolisms in the preceding 18 months and a history of monoclonal gammopathy of undetermined significance (MGUS). A CT scan showed evidence of large vessels vasculitis in the absence of circulating antineutrophil cytoplasmic antibodies. Biopsy of the toes showed evidence of light chain and immunoglobulin deposition on immunofluorescence suggesting vasculitis secondary to his haematological diagnosis of MGUS. The patient was treated with high dose glucocorticoids and immunosuppressive treatment with a significant improvement in his symptoms and features of digital ischaemia.

show abstract

Section: Discussionmentioning

confidence: 95%

“…From the literature, there have been several case reports of MGUS causing small vessel vasculitis 14 15. Umemura et al 14 describe a case of IgA-associated leukocytoclastic vasculitis associated with MGUS of an IgA lambda chain.…”

Section: Discussionmentioning

confidence: 99%

Monoclonal gammopathy of undetermined significance causing large vessel vasculitis

2022

View full text Add to dashboard Cite

show abstract

“…There are different forms of immune complex vasculitides (Table 1) (1): systemic and skin-limited variants (1) of IgA vasculitis (IgAV) (Figure 1), cryoglobulinemic vasculitis (CV), rheumatoid vasculitis (RV), lupus vasculitis (LE vasculitis), hypocomplementemic vasculitis (Figure 2), and serum sickness, as well as the provisionally defined forms of cutaneous IgM/IgG immune complex vasculitides and a recurrent macular vasculitis induced by hypergammaglobulinemia (Waldenström) (Figure 3) or by exertion or heat, or a, vasculitis in gammopathy other than cryoglobulinemic vasculitis (2,3).…”

Section: Definition and DI Erent Forms Of Immune Complex Vasculitidesmentioning

confidence: 99%

Pathophysiology and clinical manifestations of immune complex vasculitides

et al. 2023

Self Cite

View full text Add to dashboard Cite

Immune complex (IC) vasculitides present inflammations of vessel walls associated with perivascular deposition of immunoglobulins (Igs), mostly ICs. They encompass systemic and skin-limited variants of IgA vasculitis (IgAV), cryoglobulinemic vasculitis (CV), rheumatoid, lupus, and hypocomplementemic vasculitides, serum sickness cutaneous IgM/IgG (non-IgA) vasculitis, and recurrent macular (hypergammaglobulinemic or exertion-induced) vasculitis. Serum sickness and CV fulfill the criteria of a type III hypersensitivity immune reaction as large lattices of the IC precipitate at vessel walls and activate polymorphonuclear neutrophils (PMNs). Immunoglobulin-A vasculitis differs with regard to the causes of perivascular deposition of ICs since here many IgA1 molecules are hypoglycosylated (Gd-IgA1), which appears to facilitate their perivascular deposition in skin and mesangium (via e.g. CD71). The reasons for increased generation of immunoglobulins or formation of IC and their perivascular deposition in either skin or systemic organs are different and not fully explored. A common denominator of OC vasculitides is the activation of PMNs near the vessel wall via Fcy or Fcα receptors. Acute episodes of IgAV additionally require PMNs to become preactivated by IgA1 or by IC already in circulation. This intravascular priming results in increased adherence and subsequently vessel-destructive NETosis when they encounter IgA deposited at the vessel walls. Binding of IgA1 to PMNs in blood stream is associated with increased serum levels of hypogalactosidated IgA1. The characteristic clinical picture of IgAV (and also of so-called IgG/IgM vasculitis) comprises palpable or retiform purpura with a clear predilection for lower legs, probably due to stasis-related reduction in blood velocity, while in other IC vasculitides, additional factors influence the sites of vasculitides. Our knowledge of distinct forms and different pathophysiological pathways of IC vasculitides may lead to in efficacious or targeted therapies. Antibodies to complement components or intestinal budesonide for IgAV are promising agents (the latter suppresses the pathophysiologically related IgA nephropathy by reducing the generation of mucosal IgA.

show abstract

“…However, in this context, we should acknowledge that differentiation is not always possible because vessel wall infiltration may occur with late occlusive vasculopathy and intravascular thrombosis may complicate vasculitis. 6 TREATMENT While in hospital the patient remained systemically well and afebrile, and the respiratory symptoms and the rash on both ankles resolved quickly, the ischaemic changes on the feet worsened daily despite oral calcium channel blockers, intravenous iloprost therapy and sildenafil. Drugs used were oral nifedipine modified release 40 mg two times per day and oral sildenafil 25…”

Section: Differential Diagnosismentioning

confidence: 99%

Section: Differential Diagnosismentioning

confidence: 99%

An interesting case of small vessel pathology following coronavirus infection

et al. 2020

View full text Add to dashboard Cite

Vasculitis is a descriptive term for a wide variety of conditions characterised by inflammation of the blood vessels that may occur as a primary process or secondary to an underlying disease. Occlusive vasculopathy is a different clinical entity characterised by skin changes and ulceration of the lower extremities because of thrombosis of the small vessels of the dermis and is usually associated with pre-thrombotic conditions. Both conditions can be confirmed or excluded by skin biopsy. We report the case of a 63-year-old woman presenting with upper and lower respiratory tract symptoms followed by a vasculitic rash on both legs. The patient underwent extensive radiological and laboratory investigations that were negative apart from positive coronavirus OC43. A biopsy of the skin was performed. Considering the clinical presentation and the investigations performed, the diagnosis of small vessel vasculopathy following coronavirus OC43 has been suggested by the authors.

show abstract

Evidence for immunoglobulin-mediated vasculitis caused by monoclonal gammopathy in monoclonal gammopathy of unclear significance prompting oncologic treatment

Cited by 12 publications

References 11 publications

Monoclonal gammopathy of undetermined significance causing large vessel vasculitis

Monoclonal gammopathy of undetermined significance causing large vessel vasculitis

Pathophysiology and clinical manifestations of immune complex vasculitides

An interesting case of small vessel pathology following coronavirus infection

Contact Info

Product

Resources

About