Necrotizing sarcoid granulomatosis: A rarity in childhood

Abstract

Necrotizing sarcoid granulomatosis (NSG) is characterized by pulmonary nodular infiltrates, a typical histology, and a benign clinical course. The etiology and pathogenesis of the disease are still unknown. In childhood, it is extremely rare, with only three reported cases so far. Here we report on an 8-year-old girl, who to our knowledge is the youngest reported patient with NSG. The girl presented with shortness of breath and a sore throat. Chest X-ray and computed tomography (CT) scan revealed multiple nodular opacities of the lung. The symptoms and radiological findings disappeared within 6 months without any treatment. The diagnosis was based on the typical signs and symptoms of NSG and on the exclusion of other diseases. As abnormal immunological findings such as the lack of specific diphtheria antibodies in spite of vaccination against diphtheria were present, we suggest that immunologic mechanisms could play an etiologic role in the pathogenesis of NSG. In addition, the ratio of CD4+/CD8+ T-cells in the peripheral blood was significantly reduced, whereas the CD4+/CD8+ T-cell ratio in the immunohistochemical staining of the lung tissue was elevated. Since this compartmentalization is a typical finding in sarcoidosis, it supports the theory that NSG may represent a variant of sarcoidosis. However, because some characteristics of NSG are uncommon in typical sarcoidosis, NSG may also be an entity in its own right.

A 13-year-old female with shortness of breath and pleuritic chest pain

“…No typical laboratory and radiographic/functional findings can be of help in diagnosing NSG. The erythrocyte sedimentation rate was elevated in this patient, a finding present in some [9], but not all papers in the literature [6]. Pulmonary function tests showed a restrictive pattern, as in other previous cases [5,11], but this finding is not universal [4,6].…”

“…NSG in childhood, to the current authors' knowledge, has been described in literature only four times and in only two of these cases [5,6] was the pulmonary involvement isolated, as in the case described here. In the other two cases [7,8] neurogical and ophthalmological lesions were encountered as well.…”

“…The erythrocyte sedimentation rate was elevated in this patient, a finding present in some [9], but not all papers in the literature [6]. Pulmonary function tests showed a restrictive pattern, as in other previous cases [5,11], but this finding is not universal [4,6]. The CD4+/CD8+ ratio in the peripheral blood of the current patient was normal; however, in a previous case [6] this value was reported to be reduced early in the disease, but later normalised.…”

“…Hilar lymphadenopathy and extrapulmonary manifestations are more frequent in sarcoidosis [11,15], as is the presence of an elevated serum angiotensin-converting enzyme (ACE) level. In the present case, ACE could not be assessed, but in most of the previously reported cases of NSG [4][5][6]13] it was in the the normal range. In terms of outcome, both diseases generally show a benign clinical course with a favourable prognosis.…”

“…The patient reported was discharged without any treatment and 3 months later complete clinical and radiographic resolution of the disease was witnessed. A favourable outcome was also seen in an 8-yrold patient whose symptoms and radiographic findings disappeared in the absence of therapy [6]. Experience in terms of therapy is unfortunately still limited.…”