Eosinophilic granulomatosis with polyangiitis is a rare primary vasculitic disease characterized by hypereosinophilia, late onset asthma and extravascular eosinophil granulomas. We report a case presented initially with acute myocardial infarction which later only proceed with asthma, skin manifestations and peripheral neuropathy. Laboratory parameters showed hypereosinohpilia with negative perinuclear pattern of antineutrophil cytoplasmic autoantibodies (p-ANCA). Skin biopsy showed leucocytoclastic vasculitis with eosinophilic infiltration while coronary angiography was normal. The patient's symptoms improved with IV methylprednisolone, pulse cyclophosphamide and azathioprine.
Churg-Strauss syndrome, a small and medium vessel vasculitis, was first described by Churg and Strauss in 1951. It is characterised by the presence of asthma, prominent tissue and blood eosinophilia, systemic vasculitis, and pulmonary and systemic necrotising allergic granulomas. Involvement of the skin, heart and gastrointestinal tract is well documented, but ocular presentation is unusual. We describe a 40-year-old lady who presented with recurrent upper eyelid swelling due to conjunctival lesions. Although she has chronic asthma, Churg-Strauss syndrome was never suspected. The diagnosis of Churg-Strauss syndrome was only made following histological examination of the conjunctival lesions.
A 47-year-old woman with poorly controlled asthma and allergic rhinitis presented with recurrent episodes of bilateral upper eyelid swelling associated with forniceal conjunctival mass for the past 10 years. Routine blood investigations showed raised IgE levels and raised eosinophil counts. The diagnosis of Churg-Strauss syndrome (CSS) was made following biopsy of the conjunctival mass. The symptoms responded well to oral steroid treatment but recurred following cessation of the therapy. The patient was co-managed with a rheumatologist and the patient currently remains stable and is on oral Methotrexate and low dose oral steroids. Ocular involvement in CSS is unusual but this unique presentation of CSS was successfully managed, and the patient remains in remission.
We report a case of 28-year-old Malay woman who initially presented with multiple joints pain with underlying poorly controlled asthma since her childhood. She was treated as seronegative arthritis. However, the involvement of joints, asthma and high-eosinophil counts raised suspicion of Churg-Strauss syndrome. This disease is undoubtedly rare in Malaysians or even in Asian populations. After appropriate therapy was delivered for the correct diagnosis her clinical condition improved. She is currently receiving maintenance treatment.
Eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome) is a rare vasculitis in children. It commonly presents with respiratory symptoms, especially asthma, allergic rhinitis, and peripheral eosinophilia. Involvement of other systems, such as renal and cardiac, may carry a poor prognosis. Anti-neutrophil cytoplasmic antibodies have been found less frequently in children with this condition. We report a case of a 15-year-old male who presented with indurated and pruritic papules on both legs and peripheral eosinophilia without other system involvement. Histopathological findings from a skin biopsy were suggestive of eosinophilic granulomatosis with polyangiitis. Anti-neutrophil cytoplasmic antibodies were negative.
Eosinophilia is occasionally a feature of rheumatic disease. The differential diagnosis of eosinophilia includes parasitic infection, systemic vasculitides, eosinophilic arthritis, and myopathies, together with the idiopathic hypereosinophilic syndrome and malignancy. Careful evaluation of the patient should enable an accurate diagnosis to be made. Parasitic infection is the commonest cause of eosinophilia worldwide and can cause systemic disease, as illustrated by the report of Sarcocystis myositis in a group of military personnel in Malaysia. A persistent arthropathy associated with eosinophilia, but not with parasitic infection, has been reported from the far East. Drugs may also cause eosinophilia, and there has recently been much discussion of the relation between Churg-Strauss syndrome and the leukotriene antagonist zafirlukast. The present view is that reduction of steroid dose allows unmasking of previously undiagnosed Churg-Strauss syndrome. The idiopathic hypereosinophilic syndrome may represent a lymphoproliferative process; evidence for this comes from the demonstration that many patients have a clonally expanded population of aberrant T cells.