Management of Small Vessel Vasculitides

  title={Management of Small Vessel Vasculitides},
  author={Giuseppe Lopalco and Donato Rigante and Vincenzo Venerito and Giacomo Emmi and Maria Grazia Anelli and Giovanni Lapadula and Florenzo Iannone and Luca Cantarini},
  journal={Current Rheumatology Reports},
Inflammation mediated by cells of the immune system and necrosis are the most striking features observed at the histologic level in patients with vasculitides, clinical entities classified according to pathologic findings involving different organs, to etiology, or to size of vessels involved. Small vessel vasculitides (SVV) are a peculiar group of systemic disorders electively involving small intraparenchymal arteries, arterioles, capillaries, or venules and leading to different levels of… 
Pathophysiology and Principles of Management of Vasculitis and Fibromuscular Dysplasia
Prevention and management of treatment side-effects including infection, osteoporosis, peptic ulceration, diabetes, hypertension, dyslipidaemia, and mood disorders, should be incorporated into routine care.
An unusual presentation of eosinophilic angiocentric fibrosis
A case of a lady with a saddle nose deformity that had for many years been treated as granulomatosis with polyangiitis with GPA, who was found to have subglottic stenosis at the time of surgery demonstrated EAF.
Aortic valve replacement due to granulomatosis with polyangiitis: a case series
Granulomatosis with polyangiitis can be a rare cause of acute aortic valve regurgitation mimicking infective endocarditis with the need for surgical valve replacement.
IgA vasculitis (Henoch–Schönlein purpura) in children
This review focuses on the classification of IgAV/HSP and management of specific organ involvements based on recent recommendations and the new pediatric classification criteria allow pediatricians to pursue multicenter controlled studies for the unmet needs in the disease.
OP-EHCR200309 1..6
  • 2020
Delicate Role of PD-L1/PD-1 Axis in Blood Vessel Inflammatory Diseases: Current Insight and Future Significance
The most recent insights concerning the role of the PD-L1/PD-1 axis in vascular inflammation are summarized and an overview exploring the novel therapeutic approaches and challenges of manipulating these immune checkpoint proteins, PD-1 and PD- L1, for suppressing blood vessel inflammation is provided.
Safety of systemic treatments for Behçet’s syndrome
A detailed overview of the safety profile for current therapies available in the treatment of Behçet’s syndrome is provided, focusing on the main side-effects, toxicity and contraindications.
Complement blockade in ANCA-associated vasculitis: an index case, current concepts and future perspectives
A case of AAV with severe activation of cAP that developed acute renal failure and was only reversed after the addition of eculizumab, a monoclonal antibody against C5 is described.


Goodpasture's syndrome: a clinical update.
Management of noninfectious mixed cryoglobulinemia vasculitis: data from 242 cases included in the CryoVas survey.
With the use of Cox-marginal structural models, rituximab plus corticosteroids showed the greater therapeutic efficacy, but this regimen was also associated with severe infections, particularly when high doses of cortICosteroids were used, whereas death rates did not differ between the therapeutic regimens.
Rituximab for the treatment of Churg-Strauss syndrome with renal involvement.
In this pilot study, RTX was safe and successful in controlling renal disease activity in three patients with CSS and deserves further study in CSS.
Successful treatment of refractory aortitis in antineutrophil cytoplasmic antibody-associated vasculitis using tocilizumab
A 47-year-old Japanese woman developed antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) complicated by a rare combination of aortitis and hypertrophic pachymeningitis. Despite
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort.
Although EGPA relapses remain frequent, mortality has declined, at least since 1996, and multivariable analysis identified cardiomyopathy, older age, and diagnosis during or prior to 1996 as independent risk factors for death and lower eosinophil count at diagnosis as predictive of relapse.
Rituximab in the treatment of refractory or relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
Preemptive retreatment with RTX, combined with standard maintenance immunosuppressants, resulted in a sustained treatment response and rituximab proved to be an efficient and safe treatment for AN CA-positive and ANCA-negative patients.
Intravenous immunoglobulin in Henoch-Schönlein purpura complicated by cerebral hemorrhage
The case of an 8-year-old boy admitted to a district hospital with a one-week history of purpuric rash and sudden severe headache and irritability, followed by generalized seizures that required diazepam per rectum is reported.
Wegener’s Granulomatosis Strictly and Persistently Localized to One Organ Is Rare: Assessment of 16 Patients from the French Vasculitis Study Group Database
Strictly and persistently localized Wegener's granulomatosis is uncommon and optimal treatment remains to be determined.