Vasculitis: A Collection of Pearls and Myths

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Since Kussmaul and Maier described the index case of vasculitis in 1866, the field has seen many changes. What was considered for decades to be only a single disorder is now known to consist of at least 15 to 20 distinct conditions. Important strides have been made in unraveling the pathophysiology of some individual forms of vasculitis, but many mysteries remain. Over time, numerous myths and occasional pearls have arisen from the care of patients with these disorders. This collection of pearls and myths gathers lessons about the status of clinical care of vasculitis patients in the year 2008.

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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