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Treatment Mononeuropathy multiplex due to polyarteritis calls for treatment with corticosteroids and cyclophosphamide We have used intravenous methylprednisolone, 15 mg/kg, for several days, followed by oral corticosteroid treatment, and cyclophosphamide, 1 g/m2 intravenously once a month for several months, but other equivalent regimens have been suggested Azathioprine is a reasonable alternative if cyclophosphamide is not tolerated Treatment must be continued for at least several months In intractable cases and in those with systemic involvement, treatment with methotrexate may be indicated Spontaneous remission and therapeutic arrest are known, but many cases have a fatal outcome from kidney and systemic complications The infarctive nerve palsies and sensory loss of the mononeuropathies generally persist to some degree even when the systemic disease is brought under control Churg-Strauss and Hypereosinophilic Syndrome These closely related systemic illnesses involve multiple individual peripheral nerves, much as in polyarteritis The additional characteristic feature is an excess of circulating and tissue eosinophils (more so than in polyarteritis) and a tendency to involve the lungs and skin, in contrast to the typical renal and bowel infarctions of polyarteritis nodosa There is, however, a considerable degree of pathologic and clinical overlap between polyarteritis and Churg-Strauss necrotizing vasculitis with asthma and vasculitic skin lesions and a more benign asthma-eosinophilia syndrome that is less aggressive and has a greater tendency for eosinophilic in ltratration of other tissues A medication (za rlukast) that is used in Europe to treat asthma is said to have precipitated several cases of Churg-Strauss disease Rarely, the illness has been preceded by treatment with a macrolide antibiotic (eg, azithromycin), estrogen, and carbamazepine but these associations are uncertain and almost all cases are idiopathic As stated, the Churg-Strauss syndrome has many similarities to polyarteritis; indeed, the original article by Churg and Strauss in 1951 was meant in part to distinguish the two diseases on the basis of allergic granulomas and eosinophilic in ltrates that involved many organs However, similar lesions within the skin and lungs have been reported from time to time in polyarteritis nodosa Moreover, they are found in diseases such as Wegener granulomatosis, lupus erythematosus, rheumatoid disease, lymphoma, and endocarditis, but the eosinophilic in ltration is less pronounced than in Churg-Strauss syndrome We have seen other types of cutaneous disease with vasculitic mononeuritis, the most impressive being a massive leukocytoclastic vasculitis of the skin (necrotic polymorphonuclear cells surrounding venules) resulting in large con uent hemorrhagic lesions In Churg-Strauss disease, rhinitis or asthma may be present for years and only later is there marked eosinophilia and organ in ltration, particularly an eosinophilic pneumonitis The neuropathy, which then develops in approximately three-quarters of patients is usually preceded by fever and weight loss and takes the form of an acute, painful mononeuritis multiplex The granular cytoplasmic pattern of antineutrophil cytoplasmic autoantibodies (c-ANCA) of the same type that occurs in Wegener granulomatosis is found in over half of cases (see later) An infarctive vasculitis involving the vasa nervorum can be detected in sural nerve biopsy, the special histologic feature possibly being a more intense eosinophilic in ltration than is usually seen in polyarteritis nodosa The idiopathic eosinophilic syndrome comprises a heterogeneous group of disorders, the common features of which are a persistent and extreme degree of eosinophilia and eosinophilic in l-.

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tration of many organ systems Neuropathy occurs in less than half of the cases, taking the form of a painful diffuse sensorimotor syndrome with axonal damage or of a mononeuritis multiplex (see Moore et al) The pathologic appearance is one of diffuse in ltration of the nerves by eosinophils rather than a vasculitis The clinical effects are attributable to the in ltration itself or to a postulated tissue-damaging effect of the eosinophilic cell Treatment Both the Churg-Strauss and the idiopathic hypereosinophilic syndrome are treated with high doses of corticosteroids, after which the peripheral eosinophilia as well as tissue damage abate in several weeks or months Further immunosuppressive treatment in the form of azathioprine, chlorambucil, or cyclophosphamide has been used in fulminant or refractory cases, which includes most of the ones that we have seen Wegener Granulomatosis (Necrotizing Granulomatous Vasculitis) This disorder gives rise to two neuropathic syndromes one a symmetrical or asymmetrical multiple mononeuropathy indistinguishable from the other angiopathic neuropathies described earlier, the other involving the lower cranial nerves directly as they exit the skull and pass through the retropharyngeal tissues The frequency of peripheral nerve involvement in Wegener disease is much lower than in the other classic vasculitides Furthermore, the affected vessels are of a smaller caliber than in polyarteritis nodosa Nonetheless, deGroot and colleagues have emphasized in a prospective analysis of 128 patients with Wegener disease that 25 had evidence of mononeuritis multiplex with the peroneal nerve most often involved and an even greater number, with a distal polyneuropathy The proportion of their cases in which the neuropathy was the presenting or sole manifestation of disease was higher than in other similar series The nding of circulating c-ANCA is relatively speci c for Wegener granulomatosis and for Churg-Strauss disease, as mentioned earlier (Specks et al) and helps to differentiate it from polyarteritis (which displays p-ANCA) and from carcinoma, chordoma, sarcoidosis, and zoster This vasculitis and its effects on the lower cranial nerves is discussed further on page 1189 Essential Mixed Cryoglobulinemia This process may be associated with a vasculitic mononeuritis multiplex as well as a more generalized polyneuropathy In many cases, glomerulonephritis, arthralgias, and purpura are conjoined, re ecting the systemic nature of the vasculopathy, but the mononeuritis may occur in isolation The evolution in the cases under our care has been slower than in the typical vasculitic neuropathies, sometimes taking weeks or months between attacks of mononeuritis The neurologic disorder may become quiescent for long periods, during which time considerable improvement may occur There is no evident relationship between the mode of onset or severity of the neuropathy and the concentration of cryoprecipitable proteins in the serum These proteins can be detected by cooling the serum and demonstrating a precipitation of IgG and IgM proteins that redissolve upon warming to 37 C (986 F) To demonstrate this phenomenon the blood sample must be carefully transported to the laboratory in a warm water bath An association with hepatitis C is well known but most patients with cryoglobulinemia have manifested mononeuritis without this infection Treatment Garcia-Bragado and colleagues have suggested that the neuropathy can be stabilized by corticosteroids and cyclophosphamide In our experience, plasma exchange has also been bene cial, but this therapeutic measure has not been systematically tested Other aspects of the condition are discussed further on under.

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the characteristics are City, Country, Customer, and Region The illustration at right shows the result when an OLAP universe is built on top of such a BW query You can see that the cube dimension from BW results in a class in the OLAP universe (for example, dimension Customer) In addition, each characteristic in the query results in a class with dimension and detail objects Level 00 objects represent the aggregated view on this characteristic, representing the All member from the underlying cube In terms of the display attributes of a BEx query definition, we see that they are detail objects within the OLAP universe If we look at this in more detail, we see that display attributes are InfoObjects that are logically assigned or subordinated to a characteristic For example, the characteristic Customer has two attributes, Phone Copyright by SAP AG Number and Fax Number In SAP reporting tools, the display attributes can only be used in combination with the actual characteristic, which means the attribute Phone Number can only be shown in the SAP reporting tool in combination with the characteristic Customer In addition, characteristics can be defined as navigational attributes in the BW cube, which then makes these attributes available for navigational purposes in the reporting tools; navigational attributes are treated identically to a characteristic This can get a bit confusing to both the developers and also for the OLAP universe so the universes differentiate between the two and the functionality of the display versus navigational attributes goes with these objects over to the OLAP universe The following illustration shows a BW query in the BEx Query Designer The row structure includes a characteristic Customer with four display attributes: Geographical Height, Postal Code, Sector Code, and Area Code In addition, the BW query contains three navigational attributes in the rows: Regional Code, Postal Code, and Area Code.

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