Clinical and biological differences between cryoglobulinaemic and hypergammaglobulinaemic purpura in primary Sjögren's syndrome: results of a large multicentre study.

OBJECTIVES: To determine the clinical and laboratory differences between cryoglobulinaemic and hypergammaglobulinaemic purpura in primary Sjögren's syndrome (pSS), in a large Italian multicentre cohort. METHOD: Patients were selected according to the following criteria: fulfilling the American-European classification criteria for pSS, serum cryoglobulin and gammaglobulin levels evaluated, and lack of hepatitis C virus (HCV) infection. Multinomial analyses were performed by distinguishing three groups of pSS: (i) purpura associated with cryoglobulinaemic vasculitis (CV), (ii) purpura associated with hypergammaglobulinaemic vasculitis (HGV), and (iii) pSS patients without purpura (pSS controls). Patients with purpura but without cryoglobulins or hypergammaglobulinaemia were excluded. RESULTS: A total of 652 patients were enrolled in this study. Group 1/CV comprised 23/652 patients (3.53%), group 2/HGV 40/652 patients (6.13%), and group 3/pSS controls 589/652 (90.34%). The three groups were found to be significantly different from each other (post-estimation test: group 1/CV vs. group 3/pSS controls: p < 0.0001; group 1/CV vs. group 2/HGV: p = 0.0001; group 2/HGV vs. group 3/pSS controls: p = 0.0003), thus confirming the different phenotypes of purpura in pSS.Multivariate analyses revealed that peripheral neuropathy (p < 0.001), low C4 (p < 0.001), leucopaenia (p = 0.01), serum monoclonal component (p = 0.02), and the presence of anti-SSB/La antibodies (p = 0.02) characterized CV whereas rheumatoid factor (p = 0.001), leucopaenia (p = 0.01), serum monoclonal component (p = 0.01), and anti-SSA/Ro antibodies (p = 0.049) were significantly associated with HGV. Lymphoma was associated only with CV. CONCLUSIONS: HGV is a cutaneous vasculitis, related to a benign B-cell proliferation, whereas CV is a systemic immune complex-mediated vasculitis with complement activation and a higher risk of lymphoma, thus confirming CV but not HGV as a prelymphomatous condition in pSS.

[Waldenström's hyperglobulinemic purpura. Apropos of a case]. / Purpura hyperglobulinémique de Waldenström. A propos d'une observation.

The authors report the case of a thirteen-year-old girl who developed clinical manifestations suggestive of Schönlein-Henoch purpura and in whom biologic tests outruled this condition and led to the diagnosis of Waldenström hyperglobulinemic purpura. Investigations illustrated the immunologic nature of this disease, outruled an underlying disease and established the diagnosis of primary hyperglobulinemic purpura.

Purpuric rashes in cystic fibrosis.

Sixteen patients with cystic fibrosis experienced vasculitic rashes of the lower extremities that resembled hypergammaglobulinemic purpura. The rashes were associated with increased serum gammaglobulin G levels and rather severe lung disease. The rashes are probably the expression of chronic lung infection and high antigenic load in these patients. Their appearance was associated, in most cases, with poor long-term survival.

Therapy of Waldenström's benign hypergammaglobulinaemia by regular plasmapheresis.

A patient with a 20-year history of Waldenström's hypergammaglobulinaemic purpura has over that time developed increasingly troublesome clinical symptoms of purpura and joint swelling, associated with a progressive increase in the level of polyclonal IgG. These symptoms have been controlled by the use of a regular plasmapheresis regime and associated with a reduction in IgG levels.

Plasmapheresis and cold sensitivity of immunoglobulin molecules. II. A study of macroglobulinemia polyclonalis spuria and immune complex disease.

The indications for plasmapheresis in the different hyperviscosity syndromes are discussed on the basis of data on relative serum viscosity at 13 and 37 degrees C. Beside monoclonal macroglobulinemia there are conditions with a high content of polyclonal IgM and also of IgG and IgA where hyperviscosity is a dominant symptom. The importance of what may be called macroglobulinemia polyclonalis spuria is stated and a number of patients belonging to this type are discussed. Polyclonal increase in IgG, even at high levels, usually does not cause severe hyperviscosity. Case histories of patients with rheumatoid disease and marked hyperviscosity constitute one group. Another is formed by diseases leading to the development of immune complexes. Such complexes are usually caused by binding of polyclonal IgG to monoclonal IgM, but other combinations are also known. A number of clinical examples of these different disease conditions, some probably indicating intense plasmapheresis, are analyzed. The limited value of plasmapheresis alone without cytostatic treatment is stressed as regards hyperviscosity. Another indication is removal of noxious protein components.

Hypergammaglobulinemic purpura in cystic fibrosis.

Four patients are presented aged 14 to 20 years with cystic fibrosis and recurrent purpura of the legs. They have polyclonal increase of Ig but no intermediate complexes demonstrable by ultracentrifugation. The 4 patients differ from other patients with cystic fibrosis by a rapid deterioration of the clinical condition after the establishment of permanent pulmonary infection, and also by their proneness to Haemophilus influenzae infections. The clinical and laboratory findings are compatible with the diagnosis of Waldenström's hypergammaglobulinemic purpura. The heterogeneity of this syndrome is discussed.