Effect of delayed diagnosis on disease course and management of Churg–Strauss syndrome: a retrospective study

Abstract

Delayed diagnosis in patients with Churg-Strauss syndrome (CSS) is largely attributed to the variable and nonspecific presentation of the disease's initial symptoms. The aim of the study was to evaluate the effect of delayed diagnosis on the course of CSS. We conducted a retrospective study of 30 CSS patients followed up in our department. In each patient, we assessed the delay in CSS diagnosis (the time when patients already fulfilled four out of six of the American College of Rheumatology criteria and the diagnosis was not yet established), the disease activity at the time of diagnosis, and organ involvement during CSS course. A median value of 2 weeks was chosen as the cutoff point after which the diagnosis was considered as delayed. Sixteen patients were diagnosed before (group 1) and 14 patients after this cutoff point (group 2). In group 2, we found a higher Birmingham Vasculitis Activity Score at the moment of diagnosis (20.4 vs 25.1, p < 0.05) and a more severe disease course, resulting in more frequent hospitalization rates (0.64 vs 2.26/year, p < 0.00001), higher corticosteroids dose requirements (5.87 vs 11.57 mg/day converted to methylprednisolone, p < 0.0001), and additional immunosuppressive therapy administration (56.2 vs 92.8 %, p < 0.05) to maintain disease remission. All six perinuclear pattern of antineutrophil cytoplasmic antibobodies (pANCA)-positive patients (20 %) were found in group 1. Concluding, the delay in diagnosis of CSS of more than 2 weeks was found to be associated with a disease course that was more severe. The presence of the pANCA antibodies may occasionally facilitate establishment of the diagnosis.

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“…5,6 Delayed diagnosis of this uncommon disease results in progressive vital organ damage. 7 In observed patient the most sever complication was renal failure due to typical kidney involvement characterized by glomerulonephritis. Since kidney damage in ANCA-vasculitis requires therapy with cytotoxic agent [6][7][8] cyclophosphamide was used in combination with methylprednisolone i.v.…”

“…7 In observed patient the most sever complication was renal failure due to typical kidney involvement characterized by glomerulonephritis. Since kidney damage in ANCA-vasculitis requires therapy with cytotoxic agent [6][7][8] cyclophosphamide was used in combination with methylprednisolone i.v. Since used approach was effective and well tolerated the patient could reach clinical remission.…”

Progressive Small Vessel Disease Burden as a Diagnostic of Central Nervous System-Restricted Microscopic Polyangiitis: A Case Report and Review of the Literature

“…It is difficult to diagnose MPA when lesions are restricted to the CNS. The delay in the diagnosis of vasculitis would result in negative health consequences [ 12 ]. In addition, intracerebral hemorrhage in MPA sometimes can be critical and fatal [ 9 , 13 ].…”

Neurological manifestations in ANCA-associated vasculitis - assessment and treatment

“…There is no specific treatment directed solely toward the neurological symptoms. The key issue is initiating AAV treatment as early in the disease course as it is possible [15]. Thus establishing proper diagnosis remains crucial and acknowledging the neurological symptoms as a possible AAV manifestation can be helpful.…”