Search PubMed⌕ Search

Biomedical subjects

P Lamprecht

Publications and source records attributed to P Lamprecht.

54 records · Page 3Linked to original sources
PubMed1998

Mixed cryoglobulinaemia, glomerulonephritis, and ANCA: essential cryoglobulinaemic vasculitis or ANCA-associated vasculitis?

Antineutrophil cytoplasmic antibodies (ANCA) are generally believed to be strongly associated with some primary systemic vasculitides (PSV), such as Wegener's granulomatosis (WG), microscopic polyangiitis (MPA), and Churg-Strauss syndrome (CSS), which have some clinical manifestations in common and are 'pauci-immune' by immunohistology. This group of PSV has thus been termed 'ANCA-associated vasculitis' (AAV). By contrast, essential mixed cryoglobulinaemic vasculitides (EMC) are clinically heterogeneous and characterized immunologically by complement consumption and by immunocomplex depositions; they are also characteristically ANCA-negative. We report here on two SV-patients in whom the detection of cANCA (proteinase 3-ANCA in one case) in conjunction with glomerulonephritis and various extrarenal vasculitic lesions was suggestive of an AAV. However, demonstration of type II cryoglobulinaemia in conjunction with hypocomplementaemia, and histological proof of immunocomplexes in the glomerulus led to the diagnosis of an EMC, which was associated with hepatitis C virus (HCV) infection in one of the cases. Against the setting of 'false positive' cANCA in EMC, we discuss the differential diagnostic steps as well as current differential therapeutic approaches.

Adult↗
PubMedDate not supplied

Birmingham vasculitis activity score, disease extent index and complement factor C3c reflect disease activity best in hepatitis C virus-associated cryoglobulinemic vasculitis.

OBJECTIVE: Clinical measures of vasculitis activity (Birmingham vasculitis activity score = BVAS) and disease extent (Disease Extent Index = DEI), serological and immunological parameters were evaluated for the monitoring of hepatitis C virus (HCV)-associated cryoglobulinemic vasculitis (CV), treated with either cyclophosphamide or interferon-alpha 2b depending on disease severity. METHODS: Serial serum samples of 15 patients with HCV-associated CV were analyzed, and BVAS, DEI, serological and immunological parameters were recorded at diagnosis and during therapy. Eight patients were treated with interferon-alpha 2b and 7 patients with cyclophosphamide. RESULTS: A complete or partial response of the CV was seen in both treatment groups. BVAS, complement factor C3c, cryoglobulinemia, and rheumatoid factor significantly decreased in both treatment groups during 6 months (p < 0.05). DEI decrease was significant in the cyclophosphamide group (p < 0.05), and there was a trend in the interferon-alpha 2b group (p = 0.06). BVAS and DEI were significantly positively correlated, and both parameters were significantly negatively correlated with C3c levels in both treatment groups (interferon-alpha 2b/cyclophosphamide: r = -0.89, p = 0.001 versus r = -0.87, p < 0.001, respectively) whereas other parameters were not, e.g. ESR and CRP. CONCLUSIONS: Patients with different degrees of disease severity, treated with either cyclophosphamide or interferon-alpha 2b depending on their disease activity, achieved remission of their CV. BVAS, DEI and C3c were especially useful in the follow-up of HCV-associated CV. C3c correlated with BVAS and DEI during therapy and provided additional information about vasculitis activity that was not reflected by other serological or immunological parameters, e.g. ESR or CRP.

Adult↗
PubMedDate not supplied

Immune phenomena in localized and generalized Wegener's granulomatosis.

Wegener's granulomatosis (WG) is characterized by granulomatous inflammation and systemic vasculitis with a predilection for the lungs and kidneys. In most patients WG begins with a localized organ involvement of the upper respiratory tract that progresses to systemic disease (generalized WG) (1). Because of the life-threatening nature of systemic vasculitis, much effort has concentrated on elucidating the pathogenesis of the vasculitis. However, based upon a renewed interest in (innate) immune defenses against microbes, a better understanding of the chronic granulomatous inflammation may contribute to a more precise insight into the early genesis of WG. Thus, this review focuses on summarizing and discussing data for a potential pattern of disease, i.e. from localized to generalized WG with a special emphasis on granulomatous lesions of the upper respiratory tract and their alterations during the disease course.

Granulomatosis with Polyangiitis↗
PubMedDate not supplied

Prevalence of ANCA in mixed cryoglobulinemia and chronic hepatitis C virus infection.

OBJECTIVE: To determine the prevalence, target antigens and clinical associations of antineutrophil cytoplasmic antibodies (ANCA) in chronic hepatitis C without extrahepatic manifestations and in chronic hepatitis C virus (HCV)-associated mixed cryoglobulinemia (MC) in two European centers. METHODS: 50 sera from patients with chronic hepatitis C and 116 sera from HCV-associated MC were tested for cytoplasmic or perinuclear pattern (C-ANCA/P-ANCA) by indirect immunofluorescence test (IFT). ANCA target antigens were determined by enzyme-linked immunosorbent assay (ELISA). RESULTS: Clinical characteristics of the patients were not different between the two centers. Cryoglobulinemic vasculitis (CV) was biopsy-proven in about 90% of the MC patients. Two patients with HCV-associated MC and 1 patient with chronic hepatitis C had a P-ANCA. A C-ANCA was detected in 1 patient with HCV-associated MC. Eight patients with a HCV-associated MC and 5 patients with chronic hepatitis C had an ANCA either directed against bactericidal/permeability increasing protein (BPI) or cathepsin G (CG). BPI- or CG-ANCA positivity was not associated with a more severe disease course. The C-ANCA titer followed disease activity in one C-ANCA positive HCV-associated MC patient. The subspecificity of the C-ANCA was not determinable in that patient. CONCLUSION: Two new target antigens of ANCA have been identified in HCV-associated MC and chronic hepatitis C in this study. BPI-ANCA and GC-ANCA were present in about 10% of patients with HCV-associated MC or chronic hepatitis C. ELISA proved to be more sensitive in the detection of ANCA than IFT. The present study on chronic HCV infection adds to various reports on the induction of CG- and BPI-ANCA in chronic infections.

Adult↗
PubMedDate not supplied

Successful therapy of bosentan-refractory pulmonary arterial hypertension (PAH) with inhalative iloprost.

Pulmonary arterial hypertension (PAH) is a severe complication of systemic sclerosis. High vascular resistance in PAH arises from an imbalance between vasodilatory mediators (prostacyclin, NO) and vasoconstrictive mediators (endothelin, thromboxane A-2). Inhaled iloprost and the dual endothelin receptor antagonist bosentan have recently been shown to be effective in controlled clinical trials. Our case report demonstrates that patients with bosentan-refractory PAH can be successfully treated with iloprost inhalation.

Administration, Inhalation↗