Myocarditis as a Lupus Challenge: Two Case Reports

Shamma Ahmad Al-Nokhatha; Hiba Ibrahim Khogali; Maryam Abdulla Al Shehhi; Imad Tarik Jassim


J Med Case Reports. 2019;13(343) 

In This Article


We report two patients diagnosed with lupus myocarditis who had clinical improvement after starting immunosuppression. To our knowledge, the present report is one of few to demonstrate the importance of MMF in particular for remission induction. There are no published guidelines on the diagnosis of lupus myocarditis. Owing to the limited tools for detecting subclinical myocarditis, more and more cases go undetected and thereafter not managed. Current treatment strategies are based on general consensus and clinical experiences rather than randomized controlled trials.

Endomyocardial biopsy is the gold standard in diagnosing myocarditis. It is an invasive measure with a risk-related procedure and limited availability in clinical practice. Its diagnostic yield is low at only 10–20%. It can exclude other diagnoses. Therefore, myocarditis diagnosis in SLE still depends largely on clinical suspicion and ECHO findings.[5]

There are few case reports of acute myocarditis and heart failure as an initial SLE presentation. The therapeutic approach for myocarditis starts from standard measures with supportive care as first-line therapy, whereas anti-heart failure medications and additional treatment are prescribed on the basis of underlying etiology.[6] Few published data in the literature highlighted the optimal immunosuppressive medications following corticosteroid use as the mainstay of treatment for myocarditis in patients with SLE. These medications include cyclophosphamide, immunoglobulin, and plasma exchange. However, few case reports have described the use of MMF for induction of remission, although it is well known to be used as a maintenance therapy. A retrospective, multicenter study from three French university hospitals reported that 2 of 29 cases were treated with MMF as first-line immunosuppressive therapy in lupus myocarditis. The median follow-up was 37 months, and significant improvements were noted in left ventricular EF and overall cardiac recovery in patients not treated with cyclophosphamide (MMF, intravenous immunoglobulin, and plasma exchange).[1,7] A recent case series of eight patients describing the use of 18F-fluorodeoxyglucose–positron emission tomography (18F-FDG-PET)/CT in diagnosing lupus myocarditis, seven of eight patients were treated with MMF with a goal dose of 3 g/day in divided doses following steroids. Of these seven patients, two were followed with 18F-FDG-PET/CT: One patient had no myocardial uptake after 5 months, and the other one had a decrease in FDG uptake measured by a standardized uptake value factor of 3 at 13 months. Transthoracic echocardiography showed an EF of 57% (range, 50–60%) with normal wall motion in another four patients within 6–8 months. The last patient was lost to follow-up.[8] It seems to be a reasonable choice to consider MMF as a suitable induction therapy for lupus myocarditis. There are no placebo-controlled, double-blind, randomized, controlled trials specifically designed to assess the use of MMF in nonrenal lupus and myocarditis in particular.[9]

Further efforts to collect relevant data for those patients should include a multicenter registry in order to establish well-designed criteria and guidelines for an optimal treatment regimen.