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LETTER TO EDITOR
Year : 1998  |  Volume : 64  |  Issue : 5  |  Page : 249-250

Mixed connective tissue disorder : A Clarification (le)




Correspondence Address:
Vijay Gandhi


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Source of Support: None, Conflict of Interest: None


PMID: 20921786

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How to cite this article:
Gandhi V, Bhattacharya, Baruah M C. Mixed connective tissue disorder : A Clarification (le). Indian J Dermatol Venereol Leprol 1998;64:249-50

How to cite this URL:
Gandhi V, Bhattacharya, Baruah M C. Mixed connective tissue disorder : A Clarification (le). Indian J Dermatol Venereol Leprol [serial online] 1998 [cited 2019 Oct 19];64:249-50. Available from: http://www.ijdvl.com/text.asp?1998/64/5/249/4714



  To the Editor Top


In th letter to the editor (Khare KC, Khare S, Mathew G. Mixed connective tissue disorder . IJDVL 1996: 62:335) the authors have reported an intriguing if not unusual case, but appears to have unfortunately confused two very distinct diagnostic autoimmune entities viz. mixed connective tissue disease (MCTD) and overlap syndrome. We would like to stress that MCTD is a distinct entity characterised by various combinations of clinical features of SLE, systemic sclerosis and dermatomyosits and diagnositc immunological features in the form of specific antibody to U1RNP (an extractable nuclear antigen) and speckled type of ANA pattern, as first described by Sharp, et al.[1][3] In contrast, 'overlap syndrome' is a vague term describing presence of clinical and /or laboratory features of two or more distinct connective tissue disorders in the same patient. Eventually the patients with 'overlap syndrome' tend to evolve into a classical picture of a single connective tissue disease.

Therefore, with the information reported regarding the case, the authors are not in a position to correctly label the reported patient as a case of MCTD, as the essential criteria for the diagnosis (vide-supra) are not fulfilled. Further, a diagnosis of polymyositis appears to have been made empirically on the basis of 'generalised decrease in muscle power' (which by itself is unusual in PM/DM as it causes a weakness predominantly of girdle muscles). Patient's estimation of muscle enzymes (CPK, aldolase, SGOT, LDH), electromyography and muscle biopsy which are integral ARA criteria for diagnosis of polymyositis are conspicuously missing. Pulmonary symptoms (productive cough and blood stained sputum) in presence of a normal X-ray of chest have strongly been explained by authors as a feature of scleroderma. Systemic sclerosis gives rise to a 'restrictive lung disease with progressive dyspnoea. Productive cough is uncommon and haemoptysis is rare. Further, hepatosplenomegaly, as reported in the patient is not a feature either of polymyositis or systemic sclerosis. In our opinion, on basis of available information, this patient is likely to be suffering from systemic lupus erythematosus (SLE) as joint pains, fever, myositis, pulmonary symptoms and hepatosplenomegaly are all well documented features of SLE. We advise the author to get immunological investigations (ANA and to DNA) done which might help clinch the diagonsis.[4]



 
  References Top

1.Sharp CG. Mixed connective tissue disease. In : Principles of Internal Medicine 12 th Ed. Wilson DJ, Braunwaled E, Isselbacher KJ (Eds.) McGraw Hill, 1448-1449.  Back to cited text no. 1    
2.Rowell NR, Goodfield MJD. The 'connective tissue diseases' In: Textbook of Dermatology, 5th Ed . Champion RH , Burton JL, Ebling FJG (Eds.) Blackwell Scientific Publication : 2266-2268.  Back to cited text no. 2    
3.Sharp GC, Irvin WS, Taw, et al. Mixed connective tissue disease. Am J Med 1972;52;148-159.  Back to cited text no. 3    
4.Rowell NR : Dermatology, 5th Ed. Champion RH, Burton JL, Ebling FJG(Eds). Blackwell Scientific Publications: 2266-8.  Back to cited text no. 4    




 

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