Muscle-Joint Pains, Dry Mouth and Eyes? – Maybe You Have DEMS/SAPS with Thyroid Antibiodies

DEMS – dry eyes and mouth syndrome; SAPS – sicca asthenia polyalgia syndrome

These cases do not qualify for sjogren’s syndrome but have “Sicca [dryness] symptoms and “nonspecific musculoskeletal pain”. They were found to have 59.2% thyroid peroxidase antibody rate compared to 20.4% controls P= .0009.

J Rheumatol. 2009 Jul 15. [Epub ahead of print]
Increased Prevalence of Antibodies to Thyroid Peroxidase in Dry Eyes and Mouth Syndrome or Sicca Asthenia Polyalgia Syndrome.
Mavragani CP, Skopouli FN, Moutsopoulos HM  abstract here

  • Most complained more of “arthalgias” – joint aches
  • Muscle biopsy showed “presence of mild lymphocytic infiltrates in MSG biopsies without fulfilling  histopathological criteria for Sjogren’s syndrome”
  • “Abnormal SchirmerI test was evident in 9 out of the 24 patients tested (37.5%), while Rose Bengal stain of the conjunctiva, suggestive of keratoconjunctivitis sicca, was present in 9 out of the 23 patients tested (39.1%)”
  • diabetes mellitus, drug side effects, preexisting lymphoma,previous head and neck irradiation, and sarcoidosis – all which can cause dryness – were not evident.
  • Raynaud’s phenomenon (white hands in response to cold) was seen in 14.8% – no difference than seen in Sjogren’s
  • many were already on thyroid replacement.

Comment  –

  • here is another possible explanation of “Fibromyaglia”(FM) like symptoms with dryness symptoms
  • it, however, does not explain the other 40%

So how does one not miss cases of Sjogren’s syndrome?:

Early criteria were developed in 1986:
Sjögren’s syndrome. Proposed criteria for classification
Robert I. Fox, Charles A. Robinson, John G. Curd, Franklin Kozin, Francis V. Howelly
Arthritis & Rheumatism Volume 29, Issue 5, pages 577–585, May 1986  abstract here

This was modified in 2002:
Ann Rheum Dis 2002;61:554-558
Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group
C Vitali, S Bombardieri, R Jonsson, H M Moutsopoulos, E L Alexander, S E Carsons, T E Daniels, P C Fox, R I Fox, S S Kassan, S R Pillemer, N Talal, M H Weisman
free article here

Their criteria is based on a large study done previously:

Assessment of the European classification criteria for Sjogren’s syndrome in a series of clinically defined cases: results of a prospective multicentre study
Claudio Vitali et al
Annals of the Rheumatic Diseases 1996; 55: 116-121 free article here

The criteria for Sjogren’s encludes:

1) keratoconjunctivis sicca(dry eyes), as documented bySchirmer’s test (strip of paper put in lower eyelid without anesthesia – look to see if <5 mm in 5 min); Can otherwise  see it wets  rose bengal or fluorescein dye staining etc. –  As mentioned above, however 39% of DEMS cases would fail this test. Must be persistent fopr over 3 months.

2) )Objective evidence of diminished salivary gland flow(dry mouth – xerostomia) – well, dry mouth is almost universal in Fibromyalgia cases, particularly those on Amitriptyline. Allergies is common in FM; and with it, mouth breathing from allergic rhinitis –  and with that, a dry mouth. To make matters worse, the stuffy nose can be a cause of nocturnal disordered breathing which is implicated as a cause in certain cases of Fibromyalgia.

3) salivary gland imaging 

3) Minor  lip or salivary gland biopsy, obtained through normal mucosa, with the specimen abnormal- a check with a rheumatologist here has suggested that this has fallen out of favor and not very reliable.

4)Blood tests:
RA factor
ANA
?ESR

2002 review stated anti – SS antibodies -presence of Ro(SS-A) or La(SS-B) antibodies-“were present in 79 of 81 patients initially classified as having primary SS (sensitivity 97.5%), but in only seven of 121 non-SS controls (specificity 94.2%).”
A copy of the 2003 criteria:

However, a more recent article:
Beijing Da Xue Xue Bao. 2012 Feb 18;44(1):51-4.
Clinical assessment of oral diagnostic items in 2002 classification criteria for primary Sjogren’s syndrome.
Yan ZM, Wei P, Qiang L, Hua H.  abstract here

Found blood testing only “79.7% of the patients had positive anti-SSA antibody, and 75.0% of them had elevated globulin [gammaglobulin] level”

Also only “60.1% had positive parotid sialography”

They had “20.3% of the patients were finally diagnosed by performing the lower lip biopsy”.

In 1996, a chinese group also  proposed different criteria:

PRELIMINARY DIAGNOSTIC CRITERIA FOR PRIMARY SJOGREN’S SYNDROME IN CHINA
Dong Yi,Zhao Yan,Guo Xiaoping, et al.
Chinese Journal of Internal Medicine 1996-02  garbled abstract here

  • “a major with at least three of the nine minor items
  • or at least five of the minor items should be presented.

The major item is anti-SSA/SSB(+) and the minors are:
(1)dry eyes ordry mouth (3months, persistently),
(2)swolen salivary glands(recurrently or persistently),
(3)rampant dental caries,
(4)Schirmer test(5mm in 5min.)or corneal staining (+),
(5)unstimulated salivary flow (0.03ml/min) or abnormal parotid sialography,
(6)minor salivary gland biopsy (≥1focus),
(7)renal tubular acidosis,
(8)hypergammaglobuminemia (γglobulin≥30%) or hypergamma-globuminemic purpura,
(9)RF1∶20orANA1∶20.
Other connective tissue diseases,pre-existinglymphoma,AIDS,sarcoidosis,graft vs host disease must be excluded.
The criteria we proposed had a high specificity of 98.2% and sensitivity of 94.1%.

Any comments?

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