Scleroderma secondary to silica exposure. Dr. N.S.Ramburn

1. SCLERODERMA SECONDARY TO SILICA
EXPOSURE
CASE REPORT.
Dr. Nitee Sagar Ramburn
MAURITIUS
Department of Dermatology & STD.
1st Affiliated Hospital to D.M.U.

2. In recent years, there has been several cases of
connective tissue diseases, especially scleroderma,
following exposure to silica and silicone.
Below here is described a 50 year old Chinese man,
with a history of exposure to silica for several years,
developing scleroderma-like disease and pulmonary
silicosis, in a very progressive manner past 1 year.

3. Case report
 Patient Name: Wan De Xian
 Age: 50
 Gender: Male
 Chief Complaints ：
- Whole body tightness past 1 year.
- Dyspnoea for approximately 2
weeks.
- Mild difficulty in swallowing solids.
From History ：
-All started 1 year back.
-Hands, feet, face were swollen and tight feeling.
-Developed on his extremities, trunks, neck, with
Pigmentation .

4. Physical examination
-Whole body skin toughness
-Swelling of both hands with stiff movement of the
Fingers.
-Raynaud’s sign positive (+)

5. -Skin appeared shiny and thickened around the
forearm and hands.
-Flexion deformity of fingers with some ulceration
on the plantar side.

6. -Ulcerations over the right sole, posteriorly.

7. Pigmentation of skin:
-back & lower limbs-Local Temperature of the
distal end of toes 。

8. -Having difficulty talking.
-Sensation of stiffness while swallowing.
-Could not close his eyes completely.

9. Pigmentation of skin:
-body-chest wall & abdomen
-neck & trunks
-back & lower limbs-Local Temperature of the distal end of toes
decreased.

10.  2 weeks before admission:
-difficulty in breathing
-pain and swelling of fingers
-difficulty swallowing (solid food)
-dry oral mucosa
No muscle pain, or ulceration in mouth cavity.
No history of weight loss or appetite.
No past medical and family illness.
Work History
Working in glass factory since 10 years, with
constant contact to silica.

11.  Investigation
-Blood test (17/11/2010):
ESR 28↑, WBC 4,9×10⁹/L, NEU 80,3%↑,
LYMPH 11,8%. IgG 1750 mg/dl↑,
IgA 1050 mg/dl↑, CRP 11mg/L↑.
-ANA Titre 1:1000+
Type of Ana Scl-70 Antibody +ve.
 -S.Protein
 Eletrophoresis 47,3% low
 ALB 18g/dL
 α-1: 3.4, α-2: 8.0,β: 10.7, ɣ: 30.6 High.
-Blood Glucose 7,45 mmol/L
-Urine test: Protein +1.
-ECG: T-wave changes. Premature ventricular beats.
CT SCAN Abdomen: fluid in Thoracic cavity, Heart
cavity, and scrotum.
CT SCAN Chest: early stage of lung fibrosis, interstitial
pneumonia,
↑Supraclavicular and axillary Lymph Nodes, ↑fluid
level in heart cavity.
 Conclusion: findings caused by scleroderma.

12. -Barium Meal: Esophageal activity slow.
-Ultrasound: Fluid in thoracic cavity
Right side 33mm, Left side 28mm.
 22/11//2010:
Heart cavity fluid: LEFT ventricle-During
contraction: 22mm, during dilatation 15mm. RIGHT
ventricle-during contraction: 4mm, during dilation
0mm.
Right Atrium: 35×8mm.
-Cardiac Ultrasound (18/11/2010)
Range of fluid on LEFT ventricle when contraction:
19mm, when dilation: 9mm.
Range of fluid in RIGHT ventricle when
contraction: 6mm, when dilatation: 4mm.
-Biopsy report 15684 - Suggestive of scleroderma.

13. Increased and thickened dermal collagen that involves dermis; Loss of
cutaneous appendages.

14.  Treatment
 Prednisone 30mg daily
 Chinese herb(effects to activate blood
circulation)
 Furosemide 20 mg BD
 NSAIDS: Brufen 300 mg BD
 Beraprost(inhibits platelet aggregation) 40µg
TID
 Colchicine 0.5 mg BD
 Spironolactone(k+ sparing diuretic) 20 mg TDS

15. Discussion :
 Progressive systemic sclerosis - multisystem
disease.
 Characteristics:
Degeneration of the skin, heart, lungs, kidneys,
gastrointestinal tract and synovia.
 Etiology ：
 Not been clearly elucidated;
 Overproduction of qualitatively normal collagen
in the affected organs.
 Exposures to silica dust, et al
 Described often as occupational disease.

16. - Silicosis is the fibrotic disease in the lung due inhalation
of silica dust (silicone dioxide)
- Recognized as an occupational hazard
- Lung macropages exposed to silica elaborate
factors that cause chronic inflammation and
fibroblastic proliferation resulting in progressive
fibrosis.
- Silica known to be a highly immunogenic
substance and can induce an autoimmune response
leading to the development of connective tissue
diseases.

17.  PSS in most cases develops gradually after 14 or
more years of exposure to Silica.
 Our patient has been in contact to Silica for
about 10 years and PSS developed at an unusual
rapidity of a one year period.

18.  Year 2000 – Research at Epidemiology Research
Unit, Medical Bureau for Occupational Diseases,
Johannesburg, South Africa.
Relation between exposure to silica dust, the
presence of silicosis, and PSS was conducted
in white South African gold miners by means of
a case-control study.
79 cases of PSS were matched by year of birth
with an equal number of control miners
selected randomly but bearing in mind the
administrative channel through which the case
had come to be identified.
Analysis showed no association between
silicosis and PSS but did show that the
cumulative life time silica exposure was
significantly higher in the cases compared with
controls.

19.  This difference was due to a difference
in the intensity of exposure to silica
during mining service rather than a
difference in duration of service.

20.  Conclusions :
Silica therefore seems to exercise its action in those
predisposed to PSS by a mechanism other than is the
case in the pathogenesis of silicosis.
This is despite the fact that some of the autoimmune
phenomena detected in the laboratory are similar in both
PSS and silicosis.
The fact that intensity of exposure is important
suggests that doses of silica high enough to
overwhelm the lung associated lymphoid system allow
appreciable amounts of silica access to other tissues,
especially immunologically active tissues.
People in contact with silica should be monitored every
1-2 years with routine tests and CT Scan, to check the level
and amount of Silica, thus avoiding complications.

21. Thank you.