Necrotizing eosinophilic granulomatous lymphadenitis with ring- and C-shaped granulomas—an underrecognized specific manifestation of nodal Churg-Strauss syndrome

Abstract

Nodal involvement as the main or prominent clinical manifestation of Churg-Strauss Syndrome (CSS) is uncommon but is being recognized with increasing frequency. Lymph node biopsies are only obtained in CSS cases in which the classic clinical and serologic manifestations of the disease are not clear, and thus the nodal biopsy becomes crucial for recognizing a disorder associated with severe morbidity and mortality. Unfortunately, lymph node pathologists are rarely confronted with this disease, and detailed descriptions of pathologic findings in nodal CSS are scant, with only one previous detailed report. We confirmed the specificity of the findings described in the previous report, and expanded the morphologic and immunophenotypic features of nodal CSS, including a possible pathogenetic role of IgG4. The diagnosis allowed successful treatment of symptoms that had plagued the patient for over 2 years and allowed the rapid recognition of a potentially fatal acute alveolar hemorrhage that occurred after the patient underwent an aortic valve replacement for bacterial endocarditis. Treatment with anti-interleukin-5 humanized monoclonal antibody mepolizumab allowed decreasing the risk of infection and poor healing associated with highdose steroids and cyclophosphamide.

Morphologic Spectrum of Lymphadenopathy in Drug Reaction With Eosinophilia and Systemic Symptoms Syndrome

“…Hence, the clinical differential diagnosis may include connective tissue disorders, idiopathic hypereosinophilia, viral hepatitis, Churg-Strauss syndrome, and Kimura disease. 35,36 A detailed clinical history, including anti-nuclear antibody and anti-neutrophil cytoplasmic antibody serology tests, would be very helpful to reach a correct diagnosis of DRESS syndrome and exclude other entities such as idiopathic hypereosinophilia, viral hepatitis, and Kimura disease. In addition, the pathologic features of lymph node biopsy in Churg-Strauss syndrome and Kimura disease are also characteristic.…”

“…The reported pathologic features of nodal Churg-Strauss syndrome are broad, including lymphoid and plasmacytic hyperplasia, hyperplasia with eosinophilia, and hyperplasia with allergic granulomas. 36 Although the latter is the pathognomonic feature of Churg-Strauss syndrome, lymphoid hyperplasia with eosinophilia may be found in drug-induced lymphadenopathy. 36 In these cases, clinical history is of paramount importance for differentiation.…”

“…36 Although the latter is the pathognomonic feature of Churg-Strauss syndrome, lymphoid hyperplasia with eosinophilia may be found in drug-induced lymphadenopathy. 36 In these cases, clinical history is of paramount importance for differentiation. Kimura disease typically presents with eosinophilia and multiple lymphadenopathies predominantly in the head and neck region, which may be one of the differential diagnoses of DRESS syndrome clinically.…”