**5. Semi-systemic (pathologic/clinical) classification**

chemokines [54]. New results, showing the possibility that regulatory Th17 cells and corresponding cytokines (IL-17, IL-23) involved in the pathogenesis of GPS as well in WG

Vasculitis (especially Wegener´s granulomatosis) is associated with bacterial infection, in particular nasal occurence of Staphylococcus aureus. Infection may play a role in the induction of autoimmunity as well as in the effector phase of the disease. In this relation Tadema et al. [55] emphasize the role of innate immunity that is involved in the development of a Th17 driven immune response, consistent with skewing towards a Th17 T cell phenotype that has been observed in Wegener's granulomatosis. Their findings shed new light on the potential role of γ/δ T cells in host defense and inflammatory diseases, provide important new information on the pathogenic role of IL-23 and IL-1β, and underline the importance of targeting these cytokines in the development of new therapeutic interventions against many

Sutton et al. [56] demonstrated that γ/δ T cells activated by IL-1β and IL-23 are an important source of innate IL-17 and IL-21 and provide an alternative mechanism whereby IL-1 and IL-23

In other study Ooi et al. [57] suggested the importance of IL-23, a key cytokine in the induction andmaintenanceofautoimmuneresponses,inTh1responsesthatcouldplayaroleinsomeforms of glomerulonephritis especially in anti-GBM (Goodpasture) disease. This experimental work emphasizes potential mechanisms in the treatment of several forms of glomerulonephritis.

Accumulating data from animal models support a role for Th17 cells and their cytokines in various autoimmune and inflammatory processes. Emerging data from running clinical trials indicate the importance of Th17 cells in such immunological processes, too. Future studies will allow us to evaluate the role of each cytokine independently in contributing to human diseases with immune-mediated pathologies and to design optimal cytokine-targeted therapies for

After careful analysis of numerous animal studies demonstrating the importance of inflamma‐ tion in the pathogenesis of pulmonary-renal syndrome, as well as the clinical correlates demon‐ stratingactivationofthe same systems inpatientswithsystemic autoimmunity,there is a reason to hope that different modalities of anti-inflammatory treatment could ameliorate the course of the disease. The inflammatory process is however extremely complex due to its multifactorial etiologyandconsideringalsointhecontextofdifferencesamongsystems[59].Forexamplerenal failure involves complex host-kidney interactions in which the inflammatory state of the host contributes to the development of renal failure and injury of the inflamed tissue further modu‐ lates the inflammatory state of the host. One of the greatest obstacles to effective treatment is establishing the diagnosis as early as possible based on all available diagnostic procedures, including invasive ones. Earlier and more reliable identification of clinical signs and laboratory markershasbecomeanimportanttoolinrelationtoestablisheffectivetreatmentmodalities.Once the inflammatory response has been set in motion, treatment may be ineffective and could

might be used for the directed therapy of pulmonary-renal syndrome in the future.

autoimmune diseases.

these diseases [58].

may mediate autoimmune inflammation.

82 Updates in the Diagnosis and Treatment of Vasculitis

**4.10. Perspectives of treatment – modulation of inflammation**

The etiology of pulmonary-renal syndrome could be associated with variety of diseases. A possible classification based on clinical symptomatology and histopathology is described in Table 2.


**Table 2.** Etiology of pulmonary-renal syndrome
