Sarcoidosis

Case contributed by Dr Kenny Sim

Presentation

Several months of non productive cough not responding to antibiotics.

Patient Data

Age: 30
Gender: Male
Modality: X-ray

Diffuse small nodular opacities are present throughout the mid and upper zones bilaterally with areas of increased interstitial thickening. The hila are prominent bilaterally and there is widening of the right paratracheal stripe. Heart size is within normal limits. Pleural spaces are clear.

Modality: CT

 

There is bilateral upper zone predominance subcentimetre nodularity with a peri-bronchovascular, fissural and sub-pleural distribution. There is also smooth interlobular septal thickening within the upper zones bilaterally. There are more confluent regions of airspace opacity within the left upper lobe. No pleural effusions.

There is extensive mediastinal lymphadenopathy, the largest of which is a subcarinal node that measures up to 51 mm in maximum diameter. There is also bilateral symmetrical hilar lymphadenopathy. There is a conglomerate mass of enlarged porta hepatis lymph nodes that measure up to 43mm. There are also enlarged lymph nodes adjacent to the superior mesenteric artery. Bilateral axillary lymph node prominence, however these nodes do retain their normal fatty hilum.

Heart size is normal and there is no pericardial effusion appreciated on CT.

No suspicious bony lesion.

Conclusion:

Bilateral perilymphatic nodularity with an upper zone predominance, in conjunction with widespread hilar, mediastinal, porta hepatis and para-aortic lymph node enlargement is most in keeping with sarcoidosis given the chronic history provided. Lymphoma is possible but much less likely.

Modality: CT

CT Abdomen and Pelvis

Findings:

There is widespread lymph node enlargement within the abdomen, pelvis and inguinal region with the most prominent nodal enlargement seen around the porta hepatis and in the preaortic, para-aortic, common iliac and external iliac lymph node groups.

There is moderate hepatosplenomegaly with a craniocaudal length of the liver measuring 19.3 cm and the spleen measuring 16.2 cm. There is diffuse heterogeneous enhancement of the spleen. No focal liver lesions identified.

There is layering of hyperdense material within the gallbladder. The pancreas, kidneys and adrenals are normal in appearance. The bowel is unremarkable. No peritoneal free fluid or gas.

No suspicious osseous lesions.

Conclusion:

Widespread abdominal, pelvic and inguinal lymphadenopathy and moderate hepatosplenomegaly. In conjunction with the CT chest, appearances are consistent with sarcoidosis with lymphoma remaining a differential diagnosis.

Case Discussion

Serum ACE 139 U/L (ref range 8-52)

Corrected serum Ca 3.73 mmol/L (ref range 2.10-2.60)

Patient proceeded to a right cervical lymph node biopsy:

Microscopic description: Sections of lymph node show extensive non-necrotising granulomatous inflammation. Multinucleated giant cells are present. There is no evidence of malignancy.

DIAGNOSIS:  Non-necrotising granulomatous inflammation, consistent with sarcoidosis.

 

Sarcoidosis is a systemic disorder of unknown aetiology, characterised by non-caseating granulomas. Hypercalcaemia is often a feature secondary to extra-renal dysregulated production of calcitriol (1,25-VitD) by alveolar marcophages within the granulomas. Sarcoid granulomas also produce angiotensin-converting enzyme, with serum ACE levels elevated in 60% of patients. As a diagnostic test however, serum ACE lacks both sensitivity and specificity.

Common thoracic manifestations on HRCT are perilymphatic nodularity with an upper and mid zone distribution and hilar and mediastinal lymphadenopathy. The 1-2-3 sign refers to bilateral hilar and right paratracheal lymph node enlargement.

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Case Information

rID: 35528
Case created: 9th Apr 2015
Last edited: 29th Nov 2016
System: Chest
Inclusion in quiz mode: Included

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