Neurological Complications in Eosinophilic Granulomatosis with Polyangiitis (EGPA): The Roles of History and Physical Examinations in the Diagnosis of EGPA

• Published in: Internal Medicine 2017/11/15, Vol.56(22), pp.3003-3008
• Main Authors: Oiwa, Hiroshi, Mokuda, Sho, Matsubara, Tomoyasu, Funaki, Masamoto, Takeda, Ikuko, Yamawaki, Takemori, Kumagai, Kazuhiko, Sugiyama, Eiji
• Format: Article
• Language: English
• Subjects: Adult; Aged; ANCA; Asthma; Central nervous system; Churg-Strauss syndrome; Complications; Consultation; Edema; eosinophilic granulomatosis with polyangiitis; Exanthema; Extremities; Female; Fever; Granulomatosis; Granulomatosis with Polyangiitis - epidemiology; Humans; Internal medicine; Lesions; Leukocytes (eosinophilic); Male; Medical personnel; Middle Aged; Nervous System Diseases - epidemiology; Neurological complications; Original; peripheral neuropathy; Peroneal nerve; Physical Examination; Physical examinations; Retrospective Studies; Skin diseases; Time-to-Treatment
• ISSN: 0918-2918; 1349-7235
• DOI: 10.2169/internalmedicine.8457-16

Objective To investigate the clinical symptoms, the physical and neurological findings, and the clinical course of neurological complications in eosinophilic granulomatosis with polyangiitis (EGPA). Methods A retrospective chart review of EGPA cases managed by two referral hospitals was performed, with a focus on the neurological findings. The study analyzed the symptoms at the onset of EGPA and investigated their chronological relationship. The patient delay (the delay between the onset of symptoms and the initial consultation), and the physician delay (the delay from consultation to the initiation of therapy) were determined and compared. The involved nerves were identified thorough a neurological examination. The cases with central nervous system (CNS) involvement were described. Results The average duration of symptoms prior to the initiating of therapy for sensory disturbances, motor deficits, rash, edema, and fever was 23, 5, 21, 18, and 24 days, respectively. Among the EGPA-specific symptoms, sensory disturbance was often the first symptom (63%), and was usually followed by the appearance of rash within four days (63%). The average physician delay (32.9±38.3 days) was significantly longer than the average patient delay (7.9±7.8 days; p=0.010). Reduced touch sensation in the superficial peroneal area, and weakness of dorsal flexion of the first toe secondary to deep peroneal nerve involvement, were highly sensitive for identifying the presence of peripheral nerve involvement in our series of patients with EGPA. Two cases, with CNS involvement, had multiple skin lesions over their hands and feet (Janeway lesions). Conclusion Japanese physicians are not always familiar with EGPA. It is important for us to consider this disease, when an asthmatic patient complains about the new onset of an abnormal sensation in the distal lower extremities, which is followed several days later by rash.