MyPACS.net: Radiology Teaching Files > Case 48156371

Never visited SARCOIDOSIS
Contributed by: Rebecca Kessler, Resident, Brooke Army Medical Center, Texas, USA.
History:

20 something year old male

 

Images:[small]larger

Fig. 1: Chest radiograph demonstrates bilateral hilar fullness concerning for lymphadenopathy.

Fig. 2: CT demonstrates mediastinal and hilar lymphadenopathy.

Fig. 3: CT demonstrates mediastinal and hilar lymphadenopathy.

Fig. 4: Centrilobular and perilymphatic pulmonary nodules.

Fig. 5: Centrilobular and perilymphatic pulmonary nodules.

Fig. 6: From Radiographics

Fig. 7: pulmonary sarcoidosis show a fibrotic-cicatricial pattern of disease, with multiple lesions in a peribronchovascular distribution. Radiographics

Fig. 8: Axial high-resolution CT scan shows multiple micronodules with a peribronchovascular distribution in both lungs, predominantly in the upper and middle lobes. One cluster of nodules in the periphery of the left upper lobe (arrow) has coalesced to form a conglomerate lesion (macronodule). Radiographics

Fig. 9: Axial high-resolution CT scan (pulmonary parenchymal window) shows bilateral enlargement and peripheral calcification of mediastinal and hilar lymph nodes (arrows). Calcification also is visible within bilateral hilar parenchymal masses formed by multiple coalescent nodules with a peribronchovascular distribution. Histologic analysis of a specimen obtained at transbronchial biopsy of one of the masses showed sarcoid granulomas. Conglomerate masses and eggshell-like nodal calcifications also may be seen in silicosis, but they are more typically posteriorly situated within the upper lobes, not in hilar locations. Radiographics
Findings:

Chest x-ray - bilateral hilar fullness

CT

1. Mediastinal and hilar lymphadenopathy

2. Perilymphatic pulmonary nodules (peribronchiovascular and pleural)

 

Diagnosis: Sarcoidosis
Discussion:

Thoracic Sarcoidosis 'The Great Mimic'

Multi-systemic disease with variable presentation, prognosis and progression. Characterized by noncaseous epithelioid cell granulomas in absence of causitive agent such as TB, berylliosis, leprosy, hypersensitivity pneumonitis, Crohn disease, primary bilary cirrhosis and fungal disease.  Sarcoid-like reactions can also be seen in neoplasm draining to lymph nodes, chronic inflammation, post-chemo or radiation therapy.

Thoracic involvement common and cause of the majority of morbidity and mortality.

90% patients have some sort of thoracic involvement.  20% develop chronic lung disease and subsequently pulmonary fibrosis

Pathogenesis:

immune-mediated multisystem disease.

Thought to be a specific environmental agent triggering an inflammatory response in genetically susceptable host.

Imaging findings:

Bilateral hilar lymphadenopathy most common radiographic finding, followed by interstitial lung disease, occasional calcification of affected lymph nodes, pleural effusions and thickening.

Interstitial lung disease to include: perilymphatic distribution of micronodules, fibrotic changes and bilater perihilar opacities

Atypical findings: masslike/alveolar opacities, honeycomb-like cysts, miliary opacities, mosaic atenuation, tracheobronchial involvement (may have obstructive ventilatory defect on PFT's),  pleural disease (effusion and/or thickening).

Diagnosis:

If biopsy of lymph nodes or parenchyma required, may do so through transbronchial, CT guided or surgical biopsy.

Staging:

Historical based off of chest radiographic findings. Siltzback classification following five stages. Staging and prognisis have not been established with CT and CT high resolution findings.

Stage 0 Normal chest xray, 5-10%

Stage 1 Lymphadenopathy only, 50%

Stage 2 Lymphadenopathy and parenchymal lung disease, 25-30%

Stage 3 Parenchymal lung disease, 10-12%

Stage 4 Pulmonary fibrosis, 25%

Symptoms:

50% are asymptomatic

25% cough or dyspnea, bronchial hyperreactivity

25% skin lesions (erythema nodosum)

Also can see fatigue, night sweats and weight loss

Pulmonary function tests often show restrictive ventilatory defect with decreased volumes and decreased carbon monoxide diffusing capacity.

 

Clinical course:

variable

2/3 remain stable or remission within decade of diagnosis with minimal to no recurrances/sequella

20% go on to develop chronic disease with pulmonary fibrosis

5% of sarcoidosis die from pulmonary fibrosis, cardiac or neurologic involvement

Recurrence after 1 year in remission is rare however may occur in any organ system

Poor prognosis if:

  • stage 2 or 3 pulmonary disease at the time of initil diagnosis
  • disease onset after 40 years
  • black race
  • hypercalcemia
  • splenomegaly
  • osseous involvement
  • chronic uveitis
  • lupus pernio (chronic raised indurated (hardened) lesion of the skin, often purplish in color. It resembles frostbite as it is seen on ears, cheeks, lips, nose, hands, fingers, and forehead. It is most often associated with sarcoidosis.[1]:709 Microscopically, it resembles lupus vulgaris. Biopsy shows granulomatous infiltration. From Wikipedia.)

Good prognosis ( >85% spontaneous remission rate):

  • fever
  • polyarthritis
  • erythema nodosum
  • bilateral hilar lymph node enlargement (Lofgren Syndrome)

Epidemiology:

1-40/100,000

women > men

35.5 vs 10.9 per 100,000 black to whites

Whites often present without symptoms. Blacks often have severe multisystem disease

References:

March 1995 RadioGraphics, 15, 421-437.

 

Pulmonary Sarcoidosis:Typical and Atypical Manifestations at High- Resolution CT with Pathologic Correlation1. October 2010 RadioGraphics, 30, 1567-1586. http://radiographics.rsna.org/content/30/6/1567.full.pdf+html

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Additional Details:

Case Number: 48156371Last Updated: 11-14-2010
Anatomy: Chest   Pathology: Non-Infectious Inflammatory Disease
Modality: CT, Conventional RadiographAccess Level: Readable by all users
Keywords: sarcoid, mediastinal lymphadenopathy

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