Rare manifestations of Churg–Strauss syndrome: coronary artery vasospasm, temporal artery vasculitis, and reversible monocular blindness—a case report

Abstract

Churg-Strauss syndrome (CSS) is a rare illness with clinical findings characterized by asthma, eosinophilia, and vasculitis affecting medium and small-sized arteries and veins in a variety of organs. Involvement of the temporal arteries by non-giant cell eosinophilic vasculitis in CSS is quite rare and has only been published as isolated case reports or small patient series. Myocardial infarction due to coronary artery vasospasm is an unusual manifestation of CSS. We describe a case of a 39-year-old woman who had two myocardial infarctions due to severe coronary artery vasospasm and was diagnosed with CSS based on a temporal artery biopsy. During the course of her treatment, she also had another rare manifestation of CSS, monocular blindness reversible with immunosuppressive therapy.

A case of Henoch-Schönlein purpura with cardiopulmonary involvement

“…11 Such phenomenon was observed in several other vasculitic conditions, and was reviewed in several case reports. [12][13][14] Another postulated mechanism of the myocardial ischemia in our patient could be related to the acute arterial thrombosis that was associated with the presence of antiphospholipid antibodies that were sometimes found in patients with HSP. 15 The antiphospholipid antibodies however was negative in our patient.…”

“…As with most vasculitic condition, the initial treatment for patients suffering from HSP with multi-organ involvement is high dose steroid followed by immunosuppressive therapy, as in our patient. 13 Although there is evidence on the effectiveness of steroid in treating abdominal pain and joint pain in HSP, there is no good evidence demonstrating the effectiveness of long term steroid or other immunosuppressive therapy in the treatment or prevention of nephritis in HSP patients. 14 Much less is our experience in the treatments of HSP with other organs involvement, with only case reports are available.…”

Churg-Strauss Syndrome as an Unusual Cause of Dysphagia: Case Report

“…Although dysphagia has not been described as a presenting complaint of vasculitis, it has been reported in Wegener granulomatosis [ 2 , 3 ]. In contrast to the frequent involvement of peripheral nerves, cranial neuropathy is quite rare in CSS and only a few cases of optic, oculomotor, and facial neuropathy have been reported [ 4 , 5 , 6 ]. Moreover, lower cranial nerve palsy in CSS is extremely rare [ 7 , 8 ].…”

Temporal Arteritis Revealing Antineutrophil Cytoplasmic Antibody–Associated Vasculitides: A Case–Control Study

“…Généreau et al reported that systemic necrotizing vasculitis was diagnosed on the basis of TAB findings in 1.4% of patients with suspected GCA, and on the basis of inflammation of the temporal artery in 4.5% of TAB specimens (7). Likewise, rare reports have described AAVs that were revealed by manifestations of temporal arteritis (TA-AAV), including granulomatosis with polyangiitis (GPA) (8)(9)(10), microscopic polyangiitis (MPA) (11)(12)(13), and eosinophilic granulomatosis with polyangiitis (EGPA) (14)(15)(16). Nevertheless, the therapeutic management strategy and prognosis may strongly differ between GCA and AAVs, thereby supporting the notion that these diseases should not be misdiagnosed.…”