Combined Churg–Strauss syndrome and allergic bronchopulmonary aspergillosis – case report and review of the literature

Abstract

A rare case of combined Churg-Strauss syndrome (CSS) and allergic bronchopulmonary aspergillosis (ABPA) was presented. A 41-year-old woman was diagnosed with CSS based upon asthma, eosinophilia (23%), chest radiographic findings, paranasal sinusitis, peripheral neuropathy and positive p- anti-neutrophil cytoplasmic antibodies (pANCA). The diagnosis of ABPA was established on the pathological findings of allegic mucin impaction and fungal hyphae on lung biopsy. It was further proved by positive serum IgE and IgG antibodies specific to afumigatus. The clinical investigation features were reviewed in the patients with combined CSS and ABPA. All patients had the time sequence of the development of CSS after ABPA uniformly, suggesting immunopathogenesis involving the emergence of CSS. The role of lung biopsy in the diagnosis of the condition was emphasized.

Chronic pulmonary aspergillosis may cause eosinophilic granulomatosis with polyangiitis via allergic bronchopulmonary aspergillosis

“…He had tuberculosis 7 years previously, and there was a thick wall cavity in his left upper lung. Because Asperugillus species can usually colonize this location easily, Asperugillus might have been colonized there for a long time; if so, ABPA could have occurred earlier than EGPA in our case, as in previous cases [3, 4].…”

“…There have been three previous cases of co-existing EGPA and ABPA [3–5], and allergic bronchopulmonary candidiasis has also been reported to co-exist with EGPA [6] (Table 1). In two of these three cases, ABPA was diagnosed prior to EGPA, though the interval varied.…”

“…In two of these three cases, ABPA was diagnosed prior to EGPA, though the interval varied. Ren et al reported a patient with ABPA whose radiographic condition had worsened 7 years later, with eosinophilia, paranasal sinusitis, peripheral neuropathy, and positive MPO-ANCA [3], and who was subsequently diagnosed with EGPA. Stephanes et al reported a patient who developed EGPA 17 years after the diagnosis of ABPA [4].…”

“…There are two established theories regarding the coexistence of ABPA and EGPA. One holds that the ABPA precedes the EGPA [3, 4, 6, 9]. In this case, Asperugillus that has been colonized within the airway or cavity for a long time leads to the eosinophilia that is a common pathogenesis of ABPA and EGPA.…”

Occam's razor or Hickam's dictum? Allergic bronchopulmonary aspergillosis and eosinophilic granulomatosis with polyangiitis*

“…To my knowledge, only two cases of ABPA ‘progressing’ to allergic granulomatosis and angiitis or EGPA have been reported. 1 2 The precise mechanism(s) by which ABPA and possibly other allergic mycoses could relate to a systemic vasculitis such as EGPA are unclear. Both show evidence of disordered humoral immunity, with IgE elevation in allergic mycoses and presence of ANCA in some patients with EGPA; however, these are generally IgG isotypes.…”