New Cases of an Old Disease – Acute and Accelerated

Vytaute Buroviene, MD Jurgita Zaveckiene, MD, PhD The Hospital of Lithuanian University of Health Sciences (LSMU) Kauno klinikos . No thing to disclose.

WCTI 4, Boston Learning objectives

To present two rare variants of silicosis – acute and accelerated disease; To overview typical imaging findings; To illustrate these subtypes with cases.

WCTI 4, Boston Silicosis

. Oldest recognized occupational disease. (11) . Diagnosis is not a challenge in a typical case (together with detailed occupational history). . Changes in industry – new, unexpected occupations at risk. . New diagnostic challenge – non-typical presentation, “non-typical” patient.

WCTI 4, Boston Pathogenesis

Particles of free crystalline silica < 5µm

Bronchoalveolar deposition

Engulfed by macrophages

Penetration

Interstitium, pleura, lymph nodes

WCTI 4, Boston Initial exposure to silica dust

Individual Duration of Particle size Concentration susceptibility exposure

– 5 High concentrations of silica 20 YEARS 10 - 4- 10 YEARS YEARS FEW WEEKS WEEKS FEW

Acute silicosis Accelerated silicosis Chronic silicosis

WCTI 4, Boston Silicosis patient - typical?

. Male; . Young; . Elderly; . New, non-typical . Decades of work in occupations; hazardous . Relatively short environment/typical exposure time. occupation; . No suspected . No proper use of . protection devices; . Probably a smoker; Diagnostic challenge . Already suspected Radiologist might be the first one to occupational disease. suspect an occupational disease.

WCTI 4, Boston Presentation of cases

. Three individuals: . young males; . same work environment – stone dust, volatile greases, acetone, other potentially toxic materials (company manufacturing stone concrete kitchen sinks); . No proper use of personal protection devices; . Exposure time 3-4 years. . Three different clinical and radiologic outcomes.

WCTI 4, Boston Case #1

. 33 y.o. male. . Cough and shortness of breath, night sweats. . Chest x-ray  IDL (HP?)  no occupational data for HP  CT  non- specific findings  progressive changes  biopsy  lipoproteinaceous material. . Detailed occupational history – exposure to silica dust. . Diagnosis of acute silicosis. . Cessation of exposure  worsening condition  transplantation. PA , showing reticulonodular . Exposure : 4 years. pattern, more prominent in upper zones, some fibrosis with hilar traction.

WCTI 4, Boston A Case #1

D

B

C

CT views in two planes show reticulonodular pattern (micronodules, interlobular septal thickening) with perilymphatic / random distribution; areas of ground glass (A, B). Changes predominantly in upper/middle zones (D). Hilar, mediastinal lymphadenopathy (C). WCTI 4, Boston Case #1 C

A B

Two-plane CT images showing progressive disease. A-C – CT images obtained 7 months after the onset of symptoms, D-F – 10 months after the onset of symptoms. Extensive areas of ground glass, progressive reticulation and fibrosis with architectural distortion and notable volume loss (F) are seen. Images in D-F also show bilateral loculated pneumothoraces.

D E F

. Lethal ending 14 months after the onset of symptoms. WCTI 4, Boston Acute silicosis. CT findings

. Bilateral consolidation in upper posterior lung zones (with possible punctate calcifications). . Smooth thickening of interlobular septae. . Areas of “ground glass”. . Multiple centrilobular nodules. . “Crazy paving” not that often as thought previously. . Lymphadenopathy, sometimes calcified (not necessary together with parenchymal changes).

WCTI 4, Boston Acute silicosis

. Acute silicosis (silicoproteinosis) develops after relatively short exposure to silica dust (few weeks – 5 years). . Mechanical irritation  excessive discharge of surfactant and associated lipids (16); interstitial inflammation. . Progressive shortness of breath, cough, weight loss. . Rapid progression  acute . . Typically – lethal ending in a period of ~1 year.

WCTI 4, Boston Case #2

. 30 y.o. male, smoker. . Dry cough, especially in the mornings, shortness of breath, exercise intolerance, increased sweating. . Chest x-ray  IDL suspected (). . CT  sarcoidosis/occupational lung disease. . Detailed occupational history – silica dust  diagnosis of Initial PA chest radiograph. Bilateral micronodular accelerated silicosis. dissemination, predominantly in upper zones, where . Exposure: 3 years. areas of confluent opacifications are also seen. Signs of hilar lymphadenopathy, some fibrotic changes with hilar traction.

WCTI 4, Boston B Case #2

A

C

Two-plane CT views at the time of onset of symptoms. Micronodules in centrilobular and perilymphatic distribution, interlobular septae thickening (A), areas of confluent consolidation, some fibrosis in upper zones of the (B). Prominent hilar and mediastinal lymphadenopathy (C).

WCTI 4, Boston C Case #2

A

B PA chest radiograph and two-plane CT views, obtained one year after the onset of symptoms. Progressive fibrotic changes – consistant with progressive massive fibrosis (PMF) (A, C). Markedly thickened bronchial walls, traction (A, B).

. Currently - worsening condition; patient is awaiting transplant.

WCTI 4, Boston Case #3

. 31 y.o. male, smoker. . No complaints. . Check up, because coworker was diagnosed with silicosis. . Exposure: 4 years . Pathologic findings in PA chest radiograph showing chest x-ray  suspicion micronodular dissemination, more prominant interstitial markings, signs of of silicosis lCT . hilar lymphadenopathy.

WCTI 4, Boston B Case #3

A

C

CT views in two planes show diffuse multiple micronodules in centrilobular and perilymphatic distribution, slightly more predominant in upper dorsal zones together with some interlobular septae thickening (A, B). Marked lymphadenopathy (C). Accidental finding – minimal pnemumomediastinum (B). . Patient remains asymptomatic.

WCTI 4, Boston Accelerated silicosis. CT findings

. Multiple small mostly centrilobular nodules, predominantly in upper zones. . Progressive massive fibrosis (PMF) in up to 30% of cases. . Lymphadenopathy is common, although calcification of lymph nodes is rare. . Pleural plaques in up to 50% of cases. . Areas of “ground glass”, simple and traction bronchiectasis occur less often.

WCTI 4, Boston Accelerated silicosis

. Overlapping radiologic and histologic symptoms of acute and chronic disease. . Changes appear earlier and progress more rapidly (after 4-10 years of exposure). . Usually more resemblance to chronic form. . Histopathologically similar to acute silicosis with developing alveolar lipoproteinosis and interstitial inflammation, but in addition silicotic nodules are present. (15) . Greater risk of PMF and other complications.

WCTI 4, Boston Differential diagnosis

Sarcoidosis Miliary TB

Alveolar proteinosis HP

WCTI 4, Boston Outcomes

. Silicosis is not only a chronic disease. . Acute silicosis has unfavourable prognosis and results in patient’s death within a year. Radiologic findings resemble those of alveolar proteinosis. . Accelerated silicosis may have features of both – acute and chronic forms; usually more similar to chronic form with more rapid progression. . Radiologic and clinical findings highly depend on individual organism reaction (genetics, smoking, underlying diseases) despite the same environment conditions.

WCTI 4, Boston References

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WCTI 4, Boston Contact info

Vytaute Buroviene, MD Radiologist, The Hospital of Lithuanian University of Health Sciences (LSMU) Kauno klinikos, Kaunas Lithuania. [email protected]

WCTI 4, Boston