In 1951, Churg and Strauss characterized a form of necrotizing vasculitis, with eosinophilic tissue inflammation and extravascular granulomas occurring in individuals with asthma, allergic rhinitis or sinusal polyposis (Churg-Strauss Syndrome (CSS)). This rare disease has an annual incidence of 0.5- 6.8 per million persons. Like many other vasculitides, CSS has diverse clinical manifestations and is often diagnostically challenging.We report a case of CSS in a 43-year-old female patient without pre-existing asthma, allergic rhinitis or a personal atopic history who presented with generalized lymphadenopathy.
A previously healthy 43-year-old Caucasian female presented complaining of a one-month history of dry cough and progressive generalized lymphadenopathy of two-week duration. The patient reported concurrent fever, malaise, weight loss, and night sweats. Several days prior to admission, a pruritic maculopapular rash in the lower trunk had erupted causing much discomfort. Her medical history was negative for asthma, allergic rhinitis or atopy. No prescription medication and no known drug allergy were elicited on history. Her social history and family history were unremarkable. On physical examination she was febrile (39.2° C); her other vital signs were normal. Bulky (up to 2cm in diameter), and slightly tender rubbery mobile lymph nodes were noted bilaterally in the anterior cervical chain, Waldeyer’s ring, axilla, and inguinal region and her spleen was enlarged. An erythematous maculopapular rash was noted over the lower trunk, in addition to bilateral shin petechiae. The rest of her examination was normal. Initial laboratory investigations were notable for an elevated white blood cell count (15.1×109/L; reference range 4.0–11.0 ×109/L), eosinophil count (2.1×109/L; reference range 0.0–0.7×109/L), sedimentation rate (23mm/h; reference range 0–20mm/h), alkaline phosphatase (214U/L; reference range 30–115U/L) and alanine aminotransferase (164U/L; reference range 1–40U/L). Her initial chest radiograph was normal but within a few days she developed bilateral, patchy airspace disease that corresponded to hypoxemia. This patient underwent vasculitis serology, CT chest and abdomen, bronchoscopy, and excisional lymph node biopsy. CT showed bilateral patchy ground glass opacities, mediastinal and axillary lymphadenopathy as well as diffuse adenopathy in the abdomen. Histological sections of the lymph node showed the presence of granulomas containing central eosinophils and necrosis. Multiple needle-shaped Charcot-Leyden crystals were observed. In addition, the transbronchial biopsy findings of eosinophilic pneumonia with non-necrotizing vasculitis were also compatible with the final diagnosis. Subsequent blood work revealed an elevated immunoglobulin E level (196.2 KIU/L; reference range <160KIU/L), and a positive p-ANCA (Anti-MPO 9.7 KEU/L; reference range<5.0KEU/L). This patient responded initially to treatment with oral prednisone; however, within a few weeks developed sensory and motor symptoms in her left tibial nerve distribution. Symptoms stabilized with the addition of higher dose prednisone and methotrexate.
CSS is a rare form of small vessel vasculitis of unknown etiology. It is classically characterized by asthma, tissue and blood eosinophilia, necrotizing vasculitis and a granulomatous response to eosinophilic necrosis. Rare nonasthmatic cases of CSS have been encountered in the literature as “atypical”, or “limited” forms of CSS. However, to the best of our knowledge, this is the first reported case in an individual without history of asthma manifesting the disease with generalized eosinophilic lymphadenopathy. This unusual presentation highlights the diagnostic challenge in CSS because of its protean manifestation. Furthermore, an early recognition is paramount as prompt treatment with corticosteroids can lead to rapid disease remission and limit morbidity in this otherwise progressive disease.
This case illustrates the variable clinical manifestation of CSS. The lack of asthma or atopic history does not preclude the diagnosis. Lymph node involvement as part of systemic disease or as the primary site of involvement is rare but possible.
Julie Chou, No Financial Disclosure Information; No Product/Research Disclosure Information