Myocarditis as a Lupus Challenge: Two Case Reports

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

Disclosures

J Med Case Reports. 2019;13(343) 

In This Article

Case Presentations

Patient 1

Patient 1 was a 41-year-old Emirati woman who was a housewife with known hypertension, hypothyroidism, and asthma. She presented to our hospital with a 1-month history of fever associated with chills, rigors, pleuritic chest pain, pain in the small joints of the hand, cold in the extremities, and photosensitivity. She was also noted to have a 1-year history of progressive fatigue, arthralgia, 20-kg weight loss, and intermittent low- and high-grade fever. On examination, she was febrile with a temperature of 100.76 °F (38.2 °C) and a heart rate of 105 beats/minute. Her vital signs were otherwise unremarkable. Generally, she looked pale. Head examination revealed a diffuse alopecia with no oral ulcers. Cardiac examination showed normal heart sounds without murmur. Pulmonary examination revealed normal air entry bilaterally with no added sounds. The patient had palpable cervical lymphadenopathy and no rashes. Musculoskeletal examination showed sclerodactyly, hand edema with no joint tenderness, and preserved range of motion. The patient had no Raynaud's phenomenon, splinter hemorrhages, or telangiectasia. The remainder of her examination was unremarkable. She had no relevant social or family history.

Laboratory investigations revealed pancytopenia with a white blood cell count of 1.4 × 109 cells/L, platelet count of 98 × 109/L, hemoglobin 7.8 gm/dl with low mean corpuscular volume and hematocrit, a negative Coombs test result, and high inflammatory markers (erythrocyte sedimentation rate [ESR], 80 mm/hour [normal range, 0–20]; C-reactive protein [CRP], 180 mg/L [normal range, 0–8]; and ferritin, 7654 μg/L). The patient had a normal renal function test result and mild derangement in liver function enzymes. She had mildly raised creatine kinase MB (CK-MB) at 13.8 ng/ml (normal range, 0.6–6.3 ng/ml) and troponin at 0.15 ng/ml (normal, <0.1 ng/ml), and she had a normal total CK concentration. The results of an extensive infectious screen were negative. Immunological tests showed a high positive antinuclear antibodies (ANA) titer of 1:2560 with speckled pattern, negative anti–double-stranded DNA antibodies, negative antiphospholipid antibodies, and low complement levels. Her antiribonucleoprotein antibodies, anti-Sm, and anti-Ro antibodies were positive.

A chest radiograph showed cardiomegaly without effusion or infiltrates. Abdominal ultrasound showed no intra- or extrahepatic dilation, with normal common bile duct (CBD). Computed tomography (CT) of the chest suggested mild basal lung fibrosis. Apart from T-wave inversion on anterolateral chest leads, the result of the patient's electrocardiography (ECG) was unremarkable. Echocardiography (ECHO) revealed moderate regional wall systolic dysfunction (ejection Fraction [EF], 40%) with moderate pulmonary hypertension. The result of bone marrow biopsy was consistent with normochromic anemia only.

Patient 2

Patient 2 was a previously healthy 33-year-old single Emirati woman who was referred to our hospital with suspicion of SLE for further investigations. Three months prior to presentation, she had complained of profound fatigue, widespread arthralgia, ongoing dyspnea, and chest tightness. On examination, the patient was afebrile with normal vital parameters. She appeared weak and pale. No malar rash was noted. Livedo reticularis was present on her chest and back. Cardiac examination revealed normal heart sounds without murmur. Pulmonary examination revealed bilateral decreased breath sounds with bilateral basal crackles. Lower limb edema was also noted. The remainder of her examination was unremarkable. She had no relevant social or family history.

Laboratory investigations revealed normal complete blood count and renal and liver function test results, raised inflammatory markers (ESR, 103 mm/hour; CRP, 90 mg/L), a normal cardiac profile (CKMB, 1.9 ng/ml [normal range, 0.6–6.3 ng/ml]; troponin 0.03 ng/ml [normal, < 0.1 ng/ml]), and a normal total CK level.

Immunological workup showed a high positive ANA titer (1:2560) with speckled pattern, positive anti–double-stranded DNA antibodies, negative antiphospholipid antibodies, normal complements, and positive anti-Ro/La antibodies.

Chest radiography showed cardiomegaly. CT of the chest showed pleural scarring with minimal left-sided pleural effusion in keeping with pleuritis, and ECG showed normal sinus rhythm. ECHO showed severely depressed left ventricular systolic function (EF, 35%) with traces of pericardial effusion. Cardiac magnetic resonance imaging confirmed the diagnosis of myocarditis (Figure 1). Findings of CT of the coronary arteries were normal.

Figure 1.

Cardiac magnetic resonance imaging showing subepicardial late gadolinium enhancement of the basal inferior, anteroseptal, and inferoseptal segments. The findings indicate dilated cardiomyopathy with previous myocarditis (not active anymore, indicated by lack of myocardial edema)

Management

Our two patients satisfied the criteria for mixed connective tissue disease (systemic lupus predominant) and SLE, respectively. Both of them were started on a pulsed dose of corticosteroid (500 mg intravenously for 3 days) followed by oral steroid. Full doses of hydroxychloroquine, MMF, and anti-heart failure medications as needed were also prescribed. Dramatic responses were noted in the first few weeks in terms of symptoms. The EF improved to 60% in patient 1, whereas it remained the same in patient 2 after repeating the ECHO within 1 year.

Comments

3090D553-9492-4563-8681-AD288FA52ACE

processing....