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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 8  |  Issue : 1  |  Page : 19-22

Erasmus Syndrome: Co-existence of silicosis and progressive systemic sclerosis in a 55 year old male


Department of Respiratory Medicine, J. L. N. Medical College, Ajmer, Rajasthan, India

Date of Submission26-Feb-2019
Date of Decision01-Oct-2019
Date of Acceptance01-Oct-2019
Date of Web Publication11-Feb-2020

Correspondence Address:
Dr. Ramakant Dixit
Department of Respiratory Medicine, J. L. N. Medical College, Ajmer-305001, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jacp.jacp_9_19

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  Abstract 


The Erasmus syndrome describes the association of generalised progressive scleroderma following exposure to silica with or without silicosis. We report a case of Erasmus Syndrome in a 55-year old labourer presenting with shortness of breath, arthralgia, Raynaud’s phenomenon, skin tightening and microstomia along with pulmonary arterial hypertension. Among investigations, serological markers were strongly positive, high-resolution computed tomography chest showed evidence of interstitial lung disease (ILD) with mediastinal lymphadenopathy and histopathology of skin biopsy were suggestive of systemic sclerosis. Correlating the occupational history, clinical features, haematological investigations and histological findings, a diagnosis of Erasmus syndrome was safely made.

Keywords: Progressive systemic sclerosis, scleroderma, silicosis


How to cite this article:
Jalutharia J, Dixit R. Erasmus Syndrome: Co-existence of silicosis and progressive systemic sclerosis in a 55 year old male. J Assoc Chest Physicians 2020;8:19-22

How to cite this URL:
Jalutharia J, Dixit R. Erasmus Syndrome: Co-existence of silicosis and progressive systemic sclerosis in a 55 year old male. J Assoc Chest Physicians [serial online] 2020 [cited 2020 May 27];8:19-22. Available from: http://www.jacpjournal.org/text.asp?2020/8/1/19/278124




  Introduction Top


Systemic sclerosis (SS) is an autoimmune disease that involves small arteries, micro-vessels and diffuse connective tissue and is characterised by scarring and vascular obliteration in the skin and internal organs. The association of previous exposure to silica and later development of SS was first analysed by Erasmus in 1957 and is known as Erasmus syndrome.[1] This report describes Erasmus syndrome in a 55-year old male patient having occupational exposure to silica dust and later developing interstitial lung disease (ILD) and features of SS.


  Case report Top


A 55-year-old male, ex-smoker presented with a history of progressive shortness of breath for last four years and more worsening for last three months. He also had progressive skin tightening mainly over the face and extremities for last one year with joint pain mainly involving the small joints of the upper limb (metacarpo-phalangeal and inter-phalangeal joints) since last six months but dysphasia was absent. He had occasional bluish discoloration of fingers on exposure to cold.

Occupational history revealed that he worked as a stone crusher for nearly ten years in a stone quarry and left the job four years ago. Neither any family history of similar complaints nor any history of chronic drug administration in the past was noted.

On examination, pallor, clubbing and microstomia were present. Skin fixity was evident over the hands leading to apparent clawing, skin thickening and stiffness of neck muscle [Figure 1]a. Lower eyelid could not be everted due to thickening. There was slight discoloration of fingers with small painful digital ulcers [Figure 1]b. Respiratory system examination revealed tachypnoea with end-inspiratory coarse crepitations at bilateral interscapular and infrascapular areas. Cardiovascular examination revealed normal heart rate and rhythm with normal S1 but loud S2 with no added sounds. No organomegaly or free fluid was noted in abdominal examination.
Figure 1 Photograph showing (a) tight skin of hands and (b) digital ulcers.

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Complete blood counts, urinalysis, liver and renal function tests and serum electrolytes were within normal limits. Induced sputum smear was negative for acid-fast bacilli. HIV and HbsAg serology were negative. Antinuclear antibody was highly positive (7.66 units) and Anti scl 70 was strongly positive (99.43 units).

Chest radiography and high-resolution computed tomography (HRCT) showed randomly distributed multiple well-defined dense pulmonary nodules in bilateral lung fields along with multiple calcified hilar lymph nodes. Subpleural reticulations with interstitial septal thickening, focal ground-glass opacities and areas of honey combing were also present in bilateral lower lobes suggestive of ILD [Figure 2] and [Figure 3]a and [Figure 3]b. Pulmonary function test revealed restrictive ventilatory defect (Forced Vital Capacity 77% predicted, Forced Expiratory Volume 1 79% predicted, Forced Expiratory Volume 1/Forced Vital Capacity 112). Digital skiagram of hands and knee showed calcinosis cutis [Figure 4]. Two-dimensional Echocardiography revealed mild pulmonary artery hypertension (Trivial TR 35mm of Hg). Skin biopsy was taken from right forearm that showed atrophy of epidermis and collagenisation of underlying dermis with loss of dermal appendages.
Figure 2 X-ray of chest showing bilateral nodular lesions and hilar prominence.

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Figure 3 CT scan of chest showing (a) bilateral nodular lesions with focal pleural thickening and (b) reticular fibrotic areas along with bilateral calcified hilar lymph nodes.

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Figure 4 X-ray of both hand and knee joint showing calcinosis cutis.

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Correlating the history of prolonged exposure to silica dust, clinical features of SS, positive serological markers, consistent radiological and histopathological features a final diagnosis of Erasmus syndrome was made.


  Discussion Top


Silicosis is an inflammatory disease of the lung that is characterised by irreversible lung fibrosis developing from prolonged inhalation and retention of crystalline silica. This is often associated with the development of other diseases, such as pulmonary tuberculosis, lung carcinoma and less commonly, autoimmune diseases like SS, rheumatoid arthritis and systemic lupus erythematosus.[2] Exposure to silica is known to be associated with abnormalities of the humoral and cellular immunity, hyper-gammaglobulinemia and alterations in T-helper and T-suppressor lymphocytes, leading to antinuclear antibody and rheumatoid factor positivity.[3] Based on these immunological aberrances, silica exposure has been incriminated as a cause of progressive SS that resembles idiopathic SS in all manners.[4] The case presented here also had classical features of SS with a background of significant exposure to silica duct.

Prolonged inhalation and subsequent retention of silica particles result in interaction with alveolar macrophage to produce lymphokines like interleukin-1 (IL-1) and tumour necrosis factor-alpha (TNF-α). This may stimulate collagen production directly or induce other cells like monocyte or mast cells to release factors that in turn induce chronic inflammation and fibroblastic proliferation in the lungs. After recruitment of fibroblast cells, transforming growth factor TNF-β releases to increase production of elastin and deposition of collagen which may result in interstitial fibrosis.[3] The exact mechanism of pathogenesis of scleroderma secondary to silica exposure is controversial, however this is mostly attributed to autoimmune responses as silica is highly immunogenic substance. Anti-topo I antibodies are the predominant autoantibodies present in silica-associated SS. The generation of anti-topo I antibodies in genetically susceptible individuals may depend partly on the patient’s sex and on the site of organ involvement and may be triggered by silica particles acting as an immune adjuvant.[5]Erasmus syndrome is an uncommon clinical diagnosis with only few reports from Indian literature.[6] Most of these cases are symptomatic and have similar clinical, immunological and serological features like idiopathic SS, so it is important to explore all such patients for occupational and environmental exposure which can explain the disproportionate symptoms and to have a correct diagnosis.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Erasmus LD. Scleroderma in gold miners on the Witwatersrand with particular reference to pulmonary manifestations. S Afr J Lab Clin Med 1957;3:209-31.  Back to cited text no. 1
    
2.
Koeger AC, Lang T, Alcaix D, Milleron B, Rozenberg S, Chaibi P et al. Silica-associated connective tissue disease. A study of 24 cases. Medicine (Baltimore) 1995;74:221-37.  Back to cited text no. 2
    
3.
Zaghi G, Koga F, Nisihara RM, Skare TL, Handar A, Utiyama SR et al. Autoantibodies in silicosis patients and in silica-exposed individuals. Rheumatol Int 2010;30:1071-75.  Back to cited text no. 3
    
4.
Bustin MH, Bull HA, Ziegler V, Mehlhorn J, Haustein UF, Maddison PJ et al. Silica associated systemic sclerosis is clinically, scrologically and immunologically indistinguishable from idiopathic systemic sclerosis. Br J Dermatol 1990;123:725-34.  Back to cited text no. 4
    
5.
Mc Hugh NJ, Whyte J, Harvey G, Haustein UF. Anti-topoisomerase I antibodies in silica-associated systemic sclerosis. A model for autoimmunity. Arthritis Rheum 1994; 37:1198-205.  Back to cited text no. 5
    
6.
Jain S, Joshi V, Rathore YS, Khippal N. Erasmus syndrome: Silicosis and systemic sclerosis. Indian J Occup Med 2017;21:94-6.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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