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The granulomatous disorders discussed in this review are Wegener's granulomatosis
(WG), lymphomatoid granulomatosis (polymorphic reticulosis) and "idiopathic
midline granuloma". The treatment of choice of WG is combined therapy with
corticosteroids and cyclophosphamide. Severely ill patients may be treated with
intravenous bolus infusions of cyclophosphamide. Otherwise oral administration is
used. Therapy must be adjusted according to leucocyte and thrombocyte counts. After
clinical remission cyclophosphamide must be continuedfor at least a year under hydration
sufficient to cause nycturia in order to protect the bladder mucosa. Corticosteroids
can be withdrawn 9-10 months after clinical remission. Relapse of WG can
be identified by clinical and laboratory (ESR, CRP, HB, urinary sediment) findings,
including detection of anti-neutrophil cytoplasm antibodies (ANCA). Alternate
treatment with azathioprine or trimethoprim/sulfamethoxazole may be used in
patients with localized or smoldering disease. Furthermore trimethoprimIsulfamethoxazole
may be used as adjunctive treatment. Lymphomatoid granulomatosis
appears to be a T-cell lymphoma and should be treated aggressively with combination
cytotoxic therapy and irradiation of localized manifestations. "Idiopathic midline
granuloma" does not seem to exist but appears to be either WG or lymphomatoid
granulomatosis when repeated biopsies are examined with monoclonal antibodies
and/or serum examined for ANCA.