Rhupus syndrome associated with a poor prognosis

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Rhupus syndrome associated with multiple complications : a case report from Yaoundé, Cameroon. Francky Teddy A. Endomba1,Mickael Essouma1 ,Cathy.

Rhupus syndrome associated with multiple complications : a case report from Yaoundé, Cameroon Francky Teddy A. Endomba1,Mickael Essouma1 ,Cathy Mireille P. Melong1 ,Jan René Nkeck1, Madeleine Singwe-Ngandeu1,2 1Department

of Internal Medicine, Faculty of Medicine and Biomedical Sciences, University of Yaoundé I, Yaoundé, Cameroon 2Rheumatology Unit, Yaoundé Central Hospital, Yaoundé, Cameroon

Conflict of interest None

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Plan Background Case presentation Discussion Conclusion

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Background First description of « Rhupus » by Peter Schur1 Patients satisfying both Systemic Lupus Erythematosus and Rheumatoid arthritis classification criteria2 More than 150 descriptions in literature (with some cases in Africa3) Different therapeutic approach and prognosis1,3 1. Shur PH, Cecil-Loeb Textbook of Medicine, 13th. 1971, 2. Jing Li et al. Medicine. 2014 4 3. Hachicha Imen et al. Pan African Medical Journal. 2011.

Case presentation (1/8) A 37 years old nulligravida Cameroonian Past medical history Ischemic stroke seven years earlier with right lower limb residual deficit (treatment : aspirin) Ovarian tuberculosis three years earlier which required surgical intervention Aseptic Osteonecrosis of the two femoral head three years earlier (treated by prosthesis) 5

Case presentation (2/8) Chronic, bilateral, symmetrical, inflammatory joint pain : low back pain and pain of 16 peripheral joints without distal interphalangeal joints involvement No fever, no weight loss or other general sign No signs of skin involvement (hair loss, malar rash, skin discoloration) No Raynaud’s phenomenon 6

Case presentation (3/8) Criteria for rheumatoid arthritis (Revised ACR Classification Criteria of 1987) Morning stiffness Symmetrical Arthritis of more than 3 joints (16 in our patient) with the involvement of Hand Joints Positive rheumatoid factors : 192 UI/ml

Positive anti-CCP antibodies : 15 UI/ml Disease Activity Score (DAS) 28 : 6,95 7

Case presentation (4/8) Criteria for Systemic Lupus (ACR Classification Criteria of 1997) Arthritis with morning stiffness, pericardial effusion, anemia Positive anti nuclear and anti-DNA antibodies (44 UI/l)

Disease activity (SLEDAI : Systemic Lupus Erythematosus Disease Activity Index) : 11 Biologic inflammatory syndrome : CRP at 29 mg/l and Erythrocyte Sedimentation Rate at 26 mm 8

Case presentation (5/8) Concerning the treatment Initially : Hydroxychloroquine (400 mg/d) and oral prednisone (with its adjunctive therapy) At 3 months : Adjunction of Sulfasalazine (SSZ) At 12 months : Adjunction of Azathioprine (initially at 100 mg/d) and removal of SSZ Actually : treatment by Azathioprine (50 mg/d) and Hydroxychloroquine (400 mg/d) 9

ESR and CRP Values

Case presentation (6/8) 50 45 40 35 30 25 20 15 10 5 0

ESR (mm) CRP (mg/l)

At diagnosis

At 3 months

At 9 months

At 12 months

At 20 months

At 5 years

29 26

28 22

23 34

22 24

11 42

12 45

Figure 1 : Evolution C Reactive Protein and Erythrocyte Sedimentation Rate 10

Case presentation (7/8) Complications Complete bronchial stenosis due to Mycobacterium tuberculosis one year after immunological diagnosis and treated by the following regimen 2RHEZS/1RHEZ/5RHE Severe bilateral tricompartimental knee osteoarthritis treated by chrondroitin sulfate (1000 mg/d)

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Case presentation (8/8) Complications Global heart failure with dilated cardiomyopathy and sequelae of myocardial infarction with multiple valvular involvement (treatment by diuretics and enzyme converting inhibitors)

Actually Disease Activity Score 28 of 3,34 and SLEDAI (Systemic Lupus Erythematosus Disease Activity Index) of 6 12

Discussion Rhupus syndrome : classification criteria and also organ damages pertaining to SLE and RA1 Well described association of both SLE and RA with cardiovascular diseases2 Association between tuberculosis and Systemic Lupus Erythematosus3

Treatment : Corticosteroids, antimalarial and immunosuppressive drugs4 1. Tani C. et al. Autoimmunity Reviews. 2012. 2. Agca R. et al. Annals of the Rheumatic Diseases. 2014 13 3. Arenas Miras et al. Reumatologia Clinica. 2013. 4. Hachicha Imen et al. PAMJ. 2011.

Conclusion Rhupus syndrome : distinct clinical entity or coincidence presence of RA and SLE ?

THANKS

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