2 | OBSERVATION
A 24-year-old man of Malagasy origin was admitted to the cardiology
department of Mahavoky Atsimo Mahajanga Hospital with a three-week
history of dyspnea. He reported a dry cough, night sweats, and asthenia.
Four months prior to admission, he had been hospitalized for similar
dyspnea and was diagnosed with dilated cardiomyopathy (DCM) associated
with heart failure with reduced left ventricular ejection fraction. The
etiology of DCM was not fully understood. The associated diagnosis of
pulmonary embolism was made based on biological and electrical evidence.
His other medical history included grade 2 hypertension discovered at
age 22, recurrent oral aphthosis, recurrent episodes of angina treated
in childhood, and an active smoking history of 1.5 pack-years for the
past two years. He reported no joint pain or photosensitivity. He had no
significant family history. His usual treatment consisted of enalapril
10 mg/d, atenolol 25 mg/d, dapagliflozin 10 mg/d, furosemide 20 mg/d,
and rivaroxaban 20 mg/d.
On admission, hemodynamic parameters were normal. General condition was
altered, with a performance status index of 3. Physical examination
revealed hepatojugular reflux, jugular venous distention, painless,
white, soft edema of the lower extremities, galloping sounds on cardiac
auscultation, and crackling rales at the bases of the lungs. Abdominal
palpation revealed a painless pulsating periumbilical mass. Peripheral
pulses were present and symmetric. He presented with dermographism,
painful aphthae on the inner lips and cheeks, and very painful anal
ulcerative lesions (Figure 1). The lymph nodes were clear and the rest
of the examination was unremarkable.
The blood count showed normocytic anemia at 11 g/dL. Neutrophil,
lymphocyte, eosinophil, and platelet counts were normal. The erythrocyte
sedimentation rate was 103 mm per hour. C-reactive protein was 48 mg/L.
Serum glucose, blood ionogram, transaminase levels, coagulation and
lipid profiles were normal. Serologies for HIV, hepatitis B and C, and
syphilis were negative. Sputum for tuberculosis and GenXpert were
negative. Serum creatinine was 84 µmol/L (clearance: 129 mL/min).
NT-proBNP level was 550 pg/mL.
The electrocardiogram showed sinus tachycardia, S1Q3, and left
ventricular hypertrophy. The electrical axis of the heart was normal and
there were no conduction or repolarization abnormalities.
Transthoracic echocardiography revealed global hypokinesis, dilatation
of all four chambers, functional mitral and tricuspid regurgitation, and
a decrease in left ventricular ejection fraction to 37%.
A thoraco-abdomino-pelvic CT scan with contrast injection was performed.
In the thorax, it showed a right peripheral triangular condensation with
a pleural base suggestive of pulmonary infarction, minimal right
pleurisy and cardiomegaly (Figure 2). Pulmonary infarction and pleurisy
are indirect signs of pulmonary embolism. In the abdomen, two fusiform
aneurysms were found without signs of rupture: an aneurysm of the
subrenal abdominal aorta with maximum axial dimensions of 4.1 x 3.31 cm,
vertically distributed over approximately 8.44 cm, and an aneurysm of
the SMA with axial dimensions of 2.26 x 1.94 cm (Figure 3).
The dyspnea was related to congestive heart failure on DCM. The
diagnosis of BD complicated by cardiac involvement, aneurysm of the
subrenal abdominal aorta and SMA, and right pulmonary embolism was
maintained. Skin pathergy test was negative. Human leukocyte antigen
(HLA) typing was not performed. Ophthalmologic examination (after one
week of corticosteroid therapy) showed no significant pathology.
After a multidisciplinary discussion, we opted for medical management
combining corticosteroids with an immunosuppressant and avoiding
surgery. The patient received a bolus of methylprednisolone 400 mg (10
mg/kg) for three days, followed by prednisolone 40 mg (1 mg/kg/d).
Treatment with cyclophosphamide was planned but not started due to
financial constraints. Anticoagulation with rivaroxaban 20 mg/d was
continued. For the treatment of heart failure, enalapril and
dapagliflozin were maintained at the same dose with the addition of
spironolactone 25 mg/d. The doses of furosemide and atenolol were
increased to 80 mg/d and 50 mg/d, respectively.
The initial course was characterized by improvement of dyspnea and
regression of lower limb edema. After one month of corticosteroid
therapy (1 mg/kg), he had no more respiratory symptoms and his general
condition had improved. His erythrocyte sedimentation rate was 20 mm per
hour and his C-reactive protein was 10 mg/L. Corticosteroid therapy was
planned for at least six months with a tapered dose, but unfortunately
the patient was lost to follow-up.