Mixed Connective Tissue Disease Associated with Other Autoimmune Diseases as Giant Cell Myocarditis, Vitiligo and Autoimmune Hemolytic Anemia, A Multiple Autoimmune Syndrome Impossible to Type, Revised Multiple Autoimmune Syndrome Classification Extended to Type 4 Needed. About Two Cases

Abstract

Introduction: Multiple autoimmune syndrome (MAS) is defined by the presence of at least 3 autoimmune diseases in the same person. It is subdivided into 3 types. Mixed connective tissue disease (MCTD) associated to other autoimmune diseases is more and more reported and do not correspond with any type of MAS classification this is why we propose to revise multiple autoimmune syndrome classification extended to type 4 and for which we report two cases. Clinical observation: Patient 1: An 18-year-old Malian female presented to the outpatient clinic with 3-months history of pruritic achromic skin patches, Gottron's papules, polyarthralgias and exertional dyspnea. Mixed connective tissue diseases associated with vitiligo and autoimmune hemolytic anemia was raised. The mixed connective tissue diseases associating systemic lupus erythematosus (EULAR/ACR 2019), rheumatoid arthritis with subcutaneous rheumatoid nodule (EULAR/ACR 2010), systemic scleroderma (EULAR/ ACR 2013), and probable dermato-polymyositis (Bohan and Peter 1975) as well as the Kasukawa’s criteria 1988 fulfilled was considered. Vitiligo of autoimmune origin was retained because of the clinical context and the immunological disorder in this case. The positivity of the direct coombs test in a context of hemolysis allowed us to rule in the diagnosis of autoimmune hemolytic anemia. Patient 2: 30-year-old nulligest woman with no past medical history presented to the internal medicine outpatient clinic because of a gangrene of the index finger and right thumb, ecchymotic lesions over the right hand, phenomenon of Raynaud, Gottron's papules and polyarthritis that started three months ago. Suspected giant Cell myocarditis associated to CMTD with left hand dry gangrene complicating probably the vasculiratis induced by connectivitis was suggested. Suspicion giant cell myocarditis was retained because of segmental dyskinesia and impaired systolic ejection function on cardiac Doppler ultrasound, ventricular tachycardia and left bundle branch block on ECG and heart failure syndrome in a context of co-diagnosis with several autoimmune diseases and markedly improvement of the left ventricular ejection fraction with co-administration of cardiologist’prescrition and disease-modifying antirheumatic drugs. Mixed connective tissue diseases associated with dry gangrene complicating probably the vasculitis induced by connectivitis was retained based on the Kasukawa 1988 criteria and the presence of dry gangrene with the absence of other contexts that could explain the vasculitis (normal lipidogram, absence of chronic infection, iatrogeny and delebile terrain). Thus, systemic lupus erythematosus was retained in front of (EULAR/ACR 2019); rheumatoid arthritis, (EULAR/ACR 2010); probable dermatopolymyositis (Bohan and Peter 1975), and scleroderma (EULAR/ACR 2013). Conclusion: Both cases have some interesting aspects; firstly, MCTD associated with vitiligo, AHA and GCM is relatively rare and do not be occulted by Physicians who care MCTD patients, and secondly, MCTD alone or its related autoimmune diseases cannot be typed according to the current MAS classification. The limits of the current MAS classification would open the way to its revision through a systematic review and meta-analysis.