Multiorgan sarcoidosis as a diabetes insipidus mask

Abstract

Summary: Sarcoidosis is an inflammatory, multisystem disease with an undetermined etiology. The presence of noncaseating granulomas in involved organs is a characteristic pathomorphological feature. Sarcoidosis, like a chameleon, can mimic different medical conditions. Although the lungs are most commonly involved, extrapulmonary manifestations can influence any system. The clinical course of the disease may differ. Immediate initiation of glucocorticosteroid therapy is important when critical organs are impaired. A case of a patient with sarcoidosis whose first clinical symptoms were related to diabetes insipidus (DI) was presented. The diagnosis of multiple organ sarcoidosis was delayed because of an adequate response to treatment with vasopressin. The multidisciplinary diagnostic approach validated the involvement of the pituitary gland, lungs, lymph nodes, bones, and subcutaneous tissue. The presented case emphasizes the critical importance of the multifaceted differential diagnosis of patients with DI.

Learning points: Sarcoidosis usually affects the lung but can also be a multisystemic disease. The assessment of the extension of sarcoidosis remains complex. A multidisciplinary approach must identify all-organ involvement and initiate appropriate sarcoidosis treatment. Diabetes insipidus (DI) can be the first symptom of a systemic granulomatous disorder. In the differential diagnosis of DI, a comprehensive assessment of rare causes of endocrine disorders, including extrapulmonary sarcoidosis, should be considered.

Figure 1

Chest X-ray (A, posterior–anterior projection; B, lateral projection). Small nodular opacities in the right subclavian area.

Figure 2

High-resolution CT. (A) A single nodule of size 10 mm in the upper right lobe (yellow arrow). (B) Many nodular densities in the right lung. The distribution of nodules is peribronchial, perivascular, and subpleural along the interlobar horizontal and oblique fissure (red arrowheads).

Figure 3

Transbronchial lung biopsy. Hematoxylin−eosin stain. (A) magnification 100×, (B) magnification 200×; subepithelial discrete (‘sarcoid-like’") granulomas with multinucleated giant cells and epithelioid histiocytes surrounded by a thin rim of fibroblasts.

Figure 4

Core needle biopsy of the subcutaneous nodule of the left elbow fossa. Sarcoidosis involving the deeper layers of the subcutaneous tissue with the presence of multiple granulomas, Langhans giant cells, and focal inflammatory infiltration from reactive lymphocytes (A); visible infiltration of adipose tissue with its focal necrosis (B); the Langhans giant multinuclear cell (C); strong positive immunohistochemical reaction with CD163 marking macrophages/histiocytes forming granulomas (D).

Figure 5

Brain magnetic resonance (A and B, sagittal scan; C, axial scan). Pituitary gland size 9 × 8 × 6 mm (AP × RL × HF). (A) T1-weighted without contrast-enhanced image indicates the absence of ‘bright spot’ sign in the posterior pituitary (blue arrow). (B and C) T1-weighted contrast-enhanced image indicates the contrast enhancement of the pituitary stalk with a thickening of the upper basal part (blue arrows).

Figure 6

Fluorine-18-fluorodeoxyglucose PET-CT (A, coronal scan; B, C and D, axial scans). Hypermetabolic activity in the lungs, thoracic and pelvic lymph nodes, and left hip bone (yellow arrows).