Localized versus systemic vasculitis: Diagnosis and management

Abstract

Localized vasculitis restricted to a specific anatomic site or organ is often histologically indistinguishable from more severe, systemic forms of vasculitis. By definition, localized vasculitis involves blood vessels within a confined vascular distribution or single organ without clinical evidence of generalized inflammation. Important factors that determine treatment and prognosis, in what appears initially to be a localized process, include histopathologic type, organ site, and the presence of systemic inflammatory markers and symptoms. The major issue is whether single organ vasculitis is actually an isolated form of the disease in which case surgical excision is curative, or whether the single organ involvement is simply a precursor of more threatening systemic vasculitis. The Birmingham Vasculitis Activity Score is a valuable tool to identify those patients with concurrent systemic involvement. The physician's recognition of a localized versus systemic vasculitic process is important in terms of making the correct diagnosis, prescribing treatment, and arranging appropriate clinical follow-up.

Uncommon presentations of primary systemic necrotizing vasculitides: the Great Masquerades

“…While cure can be achieved in the former with surgical resection alone, isolated vasculitis of the intestines has a more aggressive course with variable prognoses, more often than not requiring immunosuppression following surgical resection, as exemplified by our patient . As was the case in this patient, a definite diagnosis of localized GI vasculitis can be made only after excluding a systemic vasculitis or connective tissue disorder through serological tests and a diligent search for the involvement of other organ systems, and also after prolonged follow‐up surveillance, if the disease remains confined to the GI tract . This case also highlights the challenges faced by the treating physicians when the family refuses to go for the best form of therapy due to fears of side effects over and above the looming threat of death due to the disease.…”

“…48,[54][55][56] As was the case in this patient, a definite diagnosis of localized GI vasculitis can be made only after excluding a systemic vasculitis or connective tissue disorder through serological tests and a diligent search for the involvement of other organ systems, and also after prolonged follow-up surveillance, if the disease remains confined to the GI tract. 57,58 This case also highlights the challenges faced by the treating physicians when the family refuses to go for the best form of therapy due to fears of side effects over and above the looming threat of death due to the disease. The author (AS) had to sit with the family for more than 1 h to convince the mother that the therapy was important to save her life and fortunately good sense prevailed.…”

Single-organ gallbladder vasculitis: Characterization and distinction from systemic vasculitis involving the gallbladder. An analysis of 57 patients

“…46 GV has also been reported in patients with autoimmune diseases, 6,11,33,42,58 and some authors have attributed a worse prognosis to patients with GV and surrogate markers of autoimmunity. 6 Previous attempts to classify SOV were based on vessel size 49 or histologic inflammatory patterns. 7 However, the revised Chapel Hill consensus conference guidelines for nomenclature and definitions of vasculitides 30 recommend that a specific type of SOV should be designated by the name of the involved organ and vessel type (for example, GB arteritis, cutaneous arteritis) and not utilize terms used for SV; because SOV is not a systemic disease, the terms PAN or giant cell arteritis of the GB would be misleading.…”

Single-Organ Gallbladder Vasculitis

“…Previous attempts to classify SOV were based on vessel size 49 or histologic inflammatory patterns. 7 However, the revised Chapel Hill consensus conference guidelines for nomenclature and definitions of vasculitides 30 recommend that a specific type of SOV should be designated by the name of the involved organ and vessel type (for example, GB arteritis, cutaneous arteritis) and not utilize terms used for SV; because SOV is not a systemic disease, the terms PAN or giant cell arteritis of the GB would be misleading.…”