Vasculitis: A Collection of Pearls and Myths
Section snippets
Myth
Every attempt should be made to discontinue prednisone in patients who have systemic vasculitis.
Comment
Despite advances in the treatment of systemic vasculitis, several forms are likely to recur after stopping conventional therapies. For example, up to 80% of patients who have Wegener granulomatosis (WG) eventually suffer disease flares, even if their disease remissions were induced by prolonged courses of cyclophosphamide (CYC) and glucocorticoids [5], [6], [7].
Clinicians are usually successful in
Pearl
Classic polyarteritis nodosa (PAN) is often a curable illness.
Comment
In contrast to WG, GCA, and some other forms of vasculitis, classic PAN—the disorder described by Kussmaul and Maier [1]—is usually a curable disease. Approximately 50% of PAN cases can be cured with glucocorticoid treatment alone, usually in courses lasting a total of 9 to 12 months [19]. The remainder of patients who have PAN require CYC to induce disease remission. Once classic PAN is in remission, however, it usually stays there
Pearl
Leukocytosis does not occur in GCA before the institution of glucocorticoid treatment.
Comment
Despite the often dramatic other indications of systemic inflammation, the white blood cell count is usually normal before the patient starts prednisone. Nonspecific laboratory abnormalities that can accompany GCA include a normochromic normocytic anemia, thrombocytosis, polyclonal hypergammaglobulinemia, and hypoalbuminemia.
Pearl
GCA does not occur among patients younger than 50 years of age.
Comment
Biologic and
Pearl
High-dose glucocorticoids are an effective therapy for refractory cutaneous Henoch-Schönlein purpura (HSP).
Comment
HSP is usually a self-limited disorder, but not always. In a small minority of patients, palpable purpura (Fig. 8) and sometimes other major disease manifestations (joint complaints, abdominal pain) recur regularly and respond poorly to a series of conventional treatment approaches: glucocorticoids at doses of up to 60 mg/d, colchicine, dapsone, azathioprine, and others. Limited anecdotal
Pearl
Strawberry gums are a classic (if uncommon) sign of WG.
Comment
WG seems to be unique among the vasculitides in its ability to cause extensive gum inflammation, as that shown in Fig. 9. When evaluating a patient for possible WG, the most detailed part of the examination takes place above the patient's neck.
Pearl
Prognostication about renal function early in the treatment course of WG is fraught with hazard.
Comment
The kidneys are often the last organ to respond to therapy in WG. Even if aggressive treatment begins
Pearl
The Churg-Strauss syndrome (CSS) often involves lymphadenopathy.
Comment
The CSS, often included as one of the ANCA-associated vasculitides (see later discussion), overlaps in many ways with WG and microscopic polyangiitis (MPA), as recognized as early as 1954 [79]. Godman and Churg noted that these three diseases “group themselves into a compass, [ranging from] necrotizing and granulomatous processes with angiitis… to vasculitis without granulomata” (with mixed forms in between) [79].
One interesting
Pearl
Interstitial fibrosis is common pulmonary manifestation of MPA.
Comment
Interstitial lung disease is an important and underrecognized complication of vasculitis associated with ANCA directed against myeloperoxidase (MPO-ANCA) (Fig. 17) [87], [88]. MPO-ANCA can occur in WG and CSS and in MPA, but interstitial lung disease usually occurs in patients whose clinical phenotype is most consistent with MPA (Table 3). Although the chest radiologic appearance of this disorder resembles usual interstitial
Pearl
Hepatitis C–associated cryoglobulinemia can present with a pulmonary-renal syndrome.
Comment
A proliferative glomerulonephritis is a well-known complication of cryoglobulinemic vasculitis. Less familiar complications of hepatitis C virus (HCV) infections can involve the lung. Pulmonary hemorrhage and interstitial fibrosis have been reported in patients who have HCV [93]. The potential association between HCV and lung dysfunction is worth keeping in mind when the pieces of a puzzling pulmonary case do
Pearl
Takayasu arteritis can be associated with extremely high platelet counts.
Comment
In a young woman who has an extremely high platelet count, a hematocrit compatible with anemia of chronic disease, and a bone marrow examination that shows reactive hyperplasia, check to make sure her pulses are palpable in all extremities (particularly her arms). Platelet counts greater than 1 million/mL in Takayasu arteritis may be observed. Platelets are acute-phase reactants and in Takayasu arteritis they reflect the
Pearl
Any kind of ocular inflammation can occur in Cogan syndrome.
Comment
Nonsyphilitic interstitial keratitis is the classic ocular manifestation of Cogan syndrome, but just about any type of ocular inflammation can occur in this disorder. In addition to interstitial keratitis and scleritis, other ocular lesions in Cogan syndrome include conjunctivitis, episcleritis, uveitis, retinal vasculitis, orbital pseudotumor, and a pan-ophthalmitis that resembles orbital pseudotumor [97]. Patients who do not have the
Pearl
Most cases of CNS vasculitis are really vasospasm.
Comment
In recent years, an important synthesis of knowledge related to CNS vasculitis has occurred. This synthesis involves the conclusion that most cases of CNS vasculitis not documented by histopathology (the great majority of all cases in this day of multiple imaging modalities) are not vasculitis at all but rather vasospasm [100]. A host of entities known by other names are often confused with and mistreated as CNS vasculitis. These include benign
Pearl
In Behçet disease, uveitis does not resolve completely between clinical episodes.
Comment
The implications of this are that the posterior uveitis of Behçet disease must be monitored carefully and therapy stopped only after great deliberation and the experience of longitudinal follow-up. Although randomized controlled trials are still lacking, tumor necrosis factor inhibition has become the standard of care now for the treatment of posterior uveitis in Behçet disease, replacing cyclophosphamide, which
Myth
Buerger disease is a systemic vasculitis.
Comment
Although often classified as a systemic vasculitis, there are several important distinctions between thromboangiitis obliterans and other forms of vasculitis [106]. Buerger disease is characterized pathologically by a highly cellular thrombus with relative sparing of the blood vessel walls. Multinucleated giant cells can even be observed within the clot in Buerger disease. The architecture of the vascular wall is preserved, and there is no fibrinoid
Myth
Distal, symmetric polyneuropathy occurring in a patient who has longstanding rheumatoid arthritis (RA) is likely to be a vasculitic neuropathy.
Comment
The inclination is to lump most patients who have symmetrical sensory symptoms into the category of distal peripheral neuropathy, along with the 400 or so other causes of this disorder (including diabetic neuropathy and alcoholic neuropathy). In patients who have RA who are the typical substrate for rheumatoid vasculitis, however, the cause of such
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Case 9-2007: a 27 year-old woman with pain and swelling of the legs
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2016, Medicina ClinicaUrologic and male genital manifestations of granulomatosis with polyangiitis
2015, Autoimmunity ReviewsCitation Excerpt :These ulcers were usually painless, recurrent and in some cases accompanied by local edema and regional lymphadenopathy [9,12,18–23], simulating a neoplasm. In Behçet's disease (in contrast to GPA), genital ulcers are often painful and mainly located in the scrotum [24]. We found eight case reports of GPA patients with destructive urethritis [25] and two more described in a specialized series of 8 patients [12].
Update on polymyalgia rheumatica
2013, European Journal of Internal MedicineCitation Excerpt :Inflammatory markers, such as the ESR and CRP, are elevated in most patients with PMR [62]. Other laboratory abnormalities associated with active inflammation may include normochromic normocytic anemia, thrombocytosis, hypoalbuminemia, and raised α-2 globulin proteins [73]. However, in a small number of cases the ESR and, rarely, the CRP may be normal despite clinically active disease [22,78].
Imaging appearances of Buerger's disease complications in the upper and lower limbs
2012, Clinical RadiologyCitation Excerpt :In this way, Buerger's disease may resemble other forms of vasculitis, which are often heralded by musculoskeletal complaints. The arthritis disappears definitively with the appearance of ischaemic signs.13 There are currently no accepted clinical criteria for the diagnosis of Buerger's disease, however, multiple have been published 1,14–16; the most commonly accepted is by Shionoya.15