A 50-year old Chinese lady comes to your office with a Raynaud s phenomenon existing since ten years. She is a teacher, she has a negative medical history. She has no other rheumatological or internal medicine complaints. A capillaroscopy is being performed in the investigation of the Raynauds phenomenon.
The correct answer is This is a scleroderma pattern according to the Fast track algorithm
The Fast track algorithm (Smith V. et al. Autoimmun Rev. 2019 Nov;18(11):102394.) consists of three rules. More specifically rule number 1: when the density of an image is 7 or more and there are no giants, then you have a non scleroderma pattern. Rule number 2: when you have giants, or an extremely lowered density (3 or less capillaries in a linear mm) consisting of abnormal shapes then you have a scleroderma pattern. Rule number 3: all pictures who donāt belong to rule 1 or rule 2 can be automatically classified as rule 3, which is: a non scleroderma pattern. The picture shown here belongs under rule number 3 and is subsequently not a scleroderma pattern.
By Prof Maurizio Cutolo Professor of Rheumatology and Professor of Internal Medicine, Director of Laboratories for Experimental Rheumatology and Academic Division of Clinical Rheumatology Director of Postgraduate School of Rheumatology University of Genova, Italy Prof Vanessa Smith Head of Clinics, Ghent University Hospital Associate Professor of Rheumatology Ghent University, Belgium
Concerning this 50-year old Chinese, with a Raynaud s phenomenon existing since ten years and a negative medical history.
The correct answer is B. When she has no scleroderma pattern on capillaroscopy and no SSc specific antibodies then her chance to get systemic sclerosis is less than 10% over the long run according to the landmark study of Koenig et al. D. When she has no scleroderma pattern on capillaroscopy and an anti -centromere then she has less than 30 % to develop systemic sclerosis according to Koenig et al in the long run E. When she both has a scleroderma pattern and an SSc specific antibody then she has a high chance to get systemic sclerosis.
Explanation concerning A,B and E: The landmark study of Koenig et al. (Arthritis Rheum. 2008 Dec;58(12):3902-12.) nicely showed that in a patient population the Raynauds phenomenon at baseline but no other signal of a connective tissue disease that patients who have no SSc-specific antibody and no scleroderma pattern on capillaroscopy have a very low chance (1.8% over the long run) to develop SSc (table 4 in the manuscript by Koenig et al.) Hence A was not correct. Hence B was correct.
Explanation concerning E: Conversely Koeing et al showed that those with both SSc-specific antibodies (Antiātopo I , antiāRNAP III and AntiāCENP-B) and a scleroderma pattern on capillaroscopy do have very high chance to get systemic sclerosis over de long run, more specifically 79,5%. Hence E is correct.
Explanation concerning C: anti SSa is not a systemic sclerosis antibody hence this patient does not have a high chance. In fact, the landmark study of Koenig et al. (Arthritis Rheum. 2008 Dec;58(12):3902-12.) nicely showed that in a patient population the Raynauds phenomenon at baseline but no other signal of a connective tissue disease that patients who have no SSc-specific antibody and no scleroderma pattern on capillaroscopy have a very low chance (1.8% over the long run) to develop SSc (table 4 in the manuscript by Koenig et al.)
Explanation concerning D: If there is no scleroderma pattern but only an SSc specific antibody positivity then the landmark paper of Koenig show that the chance to develop SSc is only 23% over the long run. Hence it is correct to say that the chance is less than 30%.