A 45-year-old woman was diagnosed with rheumatoid arthritis 11 years ago and subsequently developed progressive systemic sclerosis, systemic lupus erythematosus, and secondary Sjögren's syndrome over the next 7 years. She fulfilled classification criteria for all four conditions. She also had hypothyroidism due to Hashimoto's thyroiditis. This represents what is believed to be the first reported case of a patient having all five of these conditions simultaneously. Despite immunosuppressive treatment, her condition deteriorated rapidly and she died.

1. case
• A 45-year-old woman initially developed seropositive erosive rheumatoid
arthritis (RA) 11 years ago. She then developed progressive systemic sclerosis
(PSS) (including pulmonary hypertension, esophageal dysfunction, cardiac
involvement and sclerodactilitis), systemic lupus erythematosus (SLE) (including
photosensitivity, nephritis, leukopenia, lymphopenia, thrombocytopenia and
Coombs positive hemolytic anemia and positive anti-dsDNA), and secondary
Sjögren's syndrome (SSS) in the last 7 years before she was admitted to our
clinic. The patient fulfilled classification criteria for RA, SLE, PSS and SSS, as
determined by American College of Rheumatology. Hypothyroidism with
positive autoantibodies due to Hashimoto's thyroiditis, the beginning of which
could not be defined, was coexistent with this overlap syndrome. In the
literature, although overlap syndromes in different combinations were reported,
we very rarely observed a complex case like this patient. In our opinion, this is
the first well-documented case of RA, PSS, SLE, SSS and Hashimoto's thyroiditis
existing together in the same patient. Although immunosuppressive therapy
was administered, the disease rapidly deteriorated and the patient died.

2. Overlap syndrome

3. • Refers to a group of conditions that have clinical features of, and
meet classification criteria for, more than one well-characterised
rheumatic disease.
• Examples of overlap syndromes in rheumatology include mixed
connective tissue disease and scleromyositis
• Diagnosis depends on which diseases the patient shows symptoms
and has positive antibodies for in their lab serology.

4. •Mixed
connective
Tissue Disease

10. •MCTD

15. Diagnostic criteria
• 4 different diagnostic criteria have been proposed
Sharp
Kasukawa
Alarcon-Segovia
Kahn
 Highest sensitivity 62% and specificity 86% with Alarcon-Segovia

16. Alarcon -Segovia
• Clinical criteria 3/5 (must have synovitis or myositis)
Edema of the hands
Synovitis
Myositis
Raynaud's phenomenon
Acrosclerosis

17. Criteria by Sharp
Major Criteria Minor Criteria
-Myositis
-pulmonary involvement
-Raynaud phenomenon or esophageal dysmotility
-Swollen hands or sclerodactyly
-high anti-U1-RNP with negative anti-Sm
 Defiinite: 4 major plus serology
Or
 3 major or 2 major and 2 minor criteria and
serology
-Alopecia
-Leukopenia
-Anemia
-Pleuritis
-Pericarditis
-Arthritis
Trigeminal Neuralgia
-Malar rash
-thrombocytopenia
-Mild Myositis

18. Classification
of
overlap Syn.
Associated with specific autoantibody profile
• Mixed Connective Tissue Disease (Anti-U1 snRNP)
• Anti- Synthetase Syndrome (Anti-tRNASynthetase)
• Polymyositis And Scleroderma (Anti-PM/Scl)
• Systemic Lupus Erythematosus and Sjögren
Syndrome (Anti- La/ SSB)
Not associated with specific autoantibody profile
• Rhupus Syndrome
• Systemic Sclerosis and Sjögren Syndrome
• Systemic Sclerosis and Rheumatoid Arthritis
• Systemic Lupus Erythematosus and Systemic
Sclerosis
• Rheumatoid Arthritis and Sjögren Syndrome
• Polymyositis and Sjögren Syndrome

19. 1st investigations to order
• FBC
• ESR
• CRP
• serum urea and creatinine

20. Investigations to consider
creatine
kinase
anti-double-
stranded DNA
Smith's
antigen
anti-SS-A and
anti-SS-B

21. Emerging tests
• anti-Ku antibodies
• antipolymyositis/scleroderma antibodies
• other antisynthetase antibodies (PL7, PL12, OJ, EJ, KS, Ha and
others)

22. Agyei
Enoch
611