Systemic sclerosis (SSc) rarely overlaps with noninfective granulomatous conditions, such as sarcoidosis or pneumoconiosis.1
A 73-year-old White man with anti-Scl70 positive SSc was evaluated for arthralgia, fever, and weight loss. A full-body computed tomography (CT) scan showed mediastinal and hilar adenopathies, together with diffuse nodules in soft tissues and retroperitoneum. An 18F-fluorodeoxyglucose positron emission tomography/CT showed an increased uptake in the hilar and mediastinal lymphadenopathies, soft tissues, and retroperitoneum (Figures 1B,C), with a “leopard man” appearance (Figure 1A). Blood cultures and bacterial and fungal serology tests (Mycobacterium, Treponema, Coxiella, Bartonella, Brucella, Aspergillus) were negative. Antineutrophil cytoplasmic antibodies and IgG4 were also negative. A mediastinoscopy-guided biopsy of the nodes documented the presence of granulomas with epithelioid cells and extensive noncaseous necrosis, whereas no neoplastic nor infectious abnormalities were detected. A diagnosis of necrotizing sarcoid granulomatosis (NSG) was made, and the patient was treated with low-dose prednisone, with prompt resolution of arthralgias and fever.
Necrotizing sarcoid granulomatosis documented by 18F-FDG PET/CT scans. (A) Diffuse disease localizations giving a “leopard man” appearance (maximal intensity projection image). (B,C) Soft tissue and retroperitoneum localizations (axial fused 18F-FDG PET/CT images). Red circles: mediastinal and hilar adenopathies; white arrows: subcutaneous depositions. CT: computed tomography; FDG: fluorodeoxyglucose; PET: positron emission tomography.
Among noninfective granulomatous conditions, NSG is a rare entity wherein the presence of necrosis may lead to misdiagnosis of infections such as tuberculosis or neoplasia, thus resulting in a delay in the start of immunosuppressive therapies.2 To our knowledge, this is the first case of NSG occurring in a patients with SSc. The possible overlap of NSG in a systemic rheumatic disease should therefore be considered, in order to avoid diagnostic and therapeutic pitfalls.
Footnotes
The authors declare no conflicts of interest relevant to this article. Institutional review board approval was not required according to the authors’ institutions. The patient provided written informed consent.
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