Page 261 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Cryoglobulinemic Vasculitis 235

Figure 3. Differential diagnosis of CV and other rheumatic diseases. In clinical practice, it is possible to
observe some clinical overlap syndromes involving CV and other autoimmune lymphoproliferative
disorders. In particular, pSS and RA may share some clinico-pathological features with CV, including a
possible association with HCV infection. However, a correct differential diagnosis may be done on the
basis of some considerations: pSS shows a typical histopathological pattern of salivary gland
involvement and specific autoantibodies anti-Ro (SSA) and La (SSB), which are rarely found in
patients with CV. Conversely, cutaneous LCV, visceral organ involvement including
glomerulonephritis, hepatitis, low C4 and HCV infection, are typically found in CV. On the other hand,
erosive symmetrical polyarthritis and serum anti-CCP characterize classic RA. Moreover, B-NHL may
complicate CV, pSS, and RA. B-NHL may be suspected after careful clinical-serological monitoring.
Finally, incomplete variants of both pSS and RA, namely isolated sicca syndrome and arthritis, can be
associated to CV in the setting of chronic HCV infection, but they do not generally present typical
clinical-serological and pathological features of pSS or RA. Abbreviations: CV, cryoglobulinemic
vasculitis; RF, rheumatoid factor; CCP, cyclic citrullinated peptide; pSS, primary Sjogren syndrome;
LCV, leukocytoclastic vasculitis; B-NHL, B-cell non-Hodgkin?s lymphoma; RA, rheumatoid arthritis.

Sicca syndrome occurs in up to one-half of patients with CV however formal criteria are
satisfied in only a minority of patients [1, 4, 12, 16]. Patients with pSS share various
symptoms with CV including purpura, xerostomia, xerophthalmia, arthralgia, and laboratory
evidence of RF seropositivity, cryoglobulinemia, and the associated complication of B-cell
lymphoma [16]. Histopathological alteration of the salivary glands and the specific
autoantibody pattern (anti-Ro/SSA-La/SSB) so noted in pSS are rarely found in patients with
MCs. Moreover, pSS is rarely complicated by chronic hepatitis, glomerulonephritis, or
concomitant HCV infection. In this regard, HCV infection should be considered an
exclusionary criterion for pSS [16]. In rare cases, often in subjects without HCV infection, the
differential diagnosis is very difficult. It therefore more appropriate to classify such patients
as CV-pSS overlap syndrome [1, 4, 12, 16]. This clinical condition shows a rather severe
clinical progression, less frequent Ro/SSA and La/SSB seropositivity, high levels of serum

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