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Necrotizing Sarcoid Granulomatosis: Clinico-Radio-Pathologic Diagnosis

Axial (A), coronal (C), and parasagittal (D) computed tomography chest maximal intensity projections revealed innumerable spiculated soft-tissue nodules throughout the lungs, varying in size from 1 to 20 mm. Although the majority are centrilobular, there are some based on the pleura and within fissures, highlighted in the parasagittal slice (D). They are well defined and not typical of hypersensitivity pneumonitis. The nodules were variably avid on positron emission tomography scan (B).

Axial (A), coronal (C), and parasagittal (D) computed tomography chest maximal intensity projections revealed innumerable spiculated soft-tissue nodules throughout the lungs, varying in size from 1 to 20 mm. Although the majority are centrilobular, there are some based on the pleura and within fissures, highlighted in the parasagittal slice (D). They are well defined and not typical of hypersensitivity pneumonitis. The nodules were variably avid on positron emission tomography scan (B).

A 30-year-old man was referred to the rheumatology outpatient department and seen urgently after developing diplopia over a 4- to 8-hour period and an isolated left fourth cranial nerve palsy associated with a bifrontal and left temporal headache and episodic vertigo. Magnetic resonance imaging of the brain at that time was unremarkable. The following week he developed intermittent “stabbing” abdominal pain and associated shoulder tip pain bilaterally, right greater than left, and tenderness over the right hypochondrium. He also reported a dry productive cough, bilateral pleuritic chest pain, and that both of his calves were “stiff and sore.” In the preceding few days he had experienced drenching night sweats. His past medical history was significant only for irritable bowel syndrome and gastroesophageal reflux. Physical examination at time of review was largely unremarkable apart from a mild right sixth nerve palsy and tender liver edge with a normal liver span of 12 cm.

Assessment

Initial blood tests revealed thrombocytosis (448/nL), monocytosis (1.2/nL), hypoalbuminemia (28 g/L), and raised inflammatory markers (erythrocyte sedimentation rate 75 mm, C-reactive protein 227mg/L). Angiotensin-converting enzyme and vitamin D were within the normal range, and cytoplasmic and perinuclear antineutrophil cytoplasmic antibodies were not detected. Viral screens were negative (Epstein-Barr, hepatitis B, hepatitis C, and human immunodeficiency virus) except for cytomegalovirus immunoglobulin G antibodies, consistent with past infection.

Computed tomography of the chest revealed innumerable spiculated soft-tissue nodules throughout the lungs, varying in size from 1 to 20 mm (Figure 1A, C, D). They did not demonstrate any clear zonal predominance. The majority of nodules were in a peribronchial distribution, with some involving the pleura and fissures. No enlarged mediastinal, hilar, or axillary lymph nodes were seen. A positron emission tomography scan was highly abnormal, revealing many of the pulmonary lesions to be variably avid (Figure 1B). Intramuscular and bilateral renal cortical fludeoxyglucose-avid lesions were also identified.

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-David John Tobias McArdle, BA (Hons), MPsych (Clin Neuro), MBBS, John Patrick McArdle, MBBS, MD, Peter Jessup, MBBS (Hons), Robin A. Harle, MBBS, Serena Parker, MBBS, FRACP

This article originally appeared in the July 2017 issue of The American Journal of Medicine.

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