Lupus presenting as myocarditis : a medical challenge

Dr Shamma Ahmad Al-Nokhatha at Department of Rheumatology, Internal Medicine, Tawam Hospital, Al Ain, United Arab Emirates and colleagues have reported two rare cases of systemic lupus erythematosus presenting as myocarditis. The case has appeared in the Journal of Medical Case Reports.

Myocarditis is an uncommon manifestation of systemic lupus erythematosus in which the clinical presentation can range from subclinical to life-threatening.

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, rigours, 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 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 oedema with no joint tenderness, and preserved range of motion. The patient had no Raynaud's phenomenon, splinter haemorrhages, 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 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 a bone marrow biopsy was consistent with normochromic anaemia 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 oedema 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. The findings of CT of the coronary arteries were normal.