Clinical and radiological features of extra-pulmonary sarcoidosis: a pictorial essay

Abstract

The aim of this manuscript is to describe radiological findings of extra-pulmonary sarcoidosis. Sarcoidosis is an immune-mediated systemic disease of unknown origin, characterized by non-caseating epitheliod granulomas. Ninety percent of patients show granulomas located in the lungs or in the related lymph nodes. However, lesions can affect any organ. Typical imaging features of liver and spleen sarcoidosis include visceromegaly, with multiple nodules hypodense on CT images and hypointense on T2-weighted MRI acquisitions. Main clinical and radiological manifestations of renal sarcoidosis are nephrolithiasis, nephrocalcinosis, and acute interstitial nephritis. Brain sarcoidosis shows multiple or solitary parenchymal nodules on MRI that enhance with a ring-like appearance after gadolinium. In spinal cord localization, MRI demonstrates enlargement and hyperintensity of spinal cord, with hypointense lesions on T2-weighted images. Skeletal involvement is mostly located in small bone, showing many lytic lesions; less frequently, bone lesions have a sclerotic appearance. Ocular involvement includes uveitis, conjunctivitis, optical nerve disease, chorioretinis. Erythema nodosum and lupus pernio represent the most common cutaneous manifestations encountered. Sarcoidosis in various organs can be very insidious for radiologists, showing different imaging features, often non-specific. Awareness of these imaging features helps radiologists to obtain the correct diagnosis.Teaching Points• Systemic sarcoidosis can exhibit abdominal, neural, skeletal, ocular, and cutaneous manifestations.• T2 signal intensity of hepatosplenic nodules may reflect the disease activity.• Heerfordt’s syndrome includes facial nerve palsy, fever, parotid swelling, and uveitis.• In the vertebrae, osteolytic and/or diffuse sclerotic lesions can be found.• Erythema nodosum and lupus pernio represent the most common cutaneous manifestations.

Not everything is as it seems: neurosarcoidosis presenting as leptomeningitis

“…The lesions can appear hyperintense in T2-weighted MRI images, which are commonly localized in the superficial and deep white matter. These imaging features may be similar to those seen in multiple sclerosis [5,13]. It is imperative to mention that ancillary investigations for neurosarcoidosis have a low sensitivity, as noted by Fritz et al; therefore, the diagnosis should be complemented with clinical findings and histopathology [14].…”

Sarcoidosis with multiple organ involvement

“…Sarcoidosis may affect virtually any organ system, although 90% of patients present with pulmonary involvement 1, 2, 3…”

“…Multiple nodules may also be found. Cutaneous lesions occur in about 20%‐30% of patients1 and may assume numerous morphologic presentations. In conclusion, the clinical course of sarcoidosis is highly variable.…”

“…Sarcoidosis may affect virtually any organ system, although 90% of patients present with pulmonary involvement. [1][2][3] Extrapulmonary disease is reported in 30% of patients, with the liver and spleen being the most frequently affected abdominal organs. Homogeneous hepatomegaly often associated with splenomegaly and enlarged lymph nodes is the typical imaging feature of abdominal sarcoidosis.…”

“…Homogeneous hepatomegaly often associated with splenomegaly and enlarged lymph nodes is the typical imaging feature of abdominal sarcoidosis. 1,3 Multiple nodules may also be found. Cutaneous lesions occur in about 20%-30% of patients 1 and may assume numerous morphologic presentations.…”