133 A young girl with lupus, recurrent pericardial effusion and cardiac tamponade. (April 2019) - Anjani, Gummadi
Abstract : Background: Pleuritis and pericarditis, with or without effusion, are the commonest pulmonary and cardiovascular manifestations in children with systemic lupus erythematosus(SLE).However, SLE presenting as an isolated pericardial effusion with cardiac tamponade is distinctly unusual. Methods: We report one such case who went on to develop recurrent episodes of pericardial effusion and tamponade. Results: A-14-year-old girl presented with high grade fever, progressive dyspnea, pedal edema, periorbital puffiness for 7 days.She also had history of malar rash and photosensitivity.There was no history of oral ulcer and hair loss.Physical examination showed a prominent malar rash;heart rate 140/min;respiratory-rate 40/min;muffled heart sounds;hepatomegaly and ascites.She was initiated on decongestive measures with furosemide and digoxin.Chest X-ray showed cardiomegaly(cardiothoracic ratio 68%);2D-echocardiography(2D-ECHO) revealed cardiomegaly, pericardial effusion and features consistent with cardiac tamponade.She underwent emergency pericardiocentesis, 400 ml of serosanguineous fluid was drained followed by prompt clinical improvement.Investigations showed hemoglobin 90 g/L; total leukocyte count 1.3 × 106/L and lymphocyte count 0.18 × 106/L;platelets of 356 × 109/L.Urinalysis and renal function tests were unremarkable.Tuberculin skin test was non-reactive.Thyroid function tests were normal.In view of febrile illness, malar rash, photosensitivity, pericardial effusion, cardiac tamponade and lymphopenia, SLE was considered.Work-up showed positive antinuclear antibody (ANA)4+diffuse ANA positivity;positive anti-double stranded DNA(anti-dsDNA)−60(normal:4.2 IU/ml);C3 51.8 mg% (normal 50–150 mg%).Skin biopsy revealed IgG and C3 deposits.Pericardial fluid analysis revealed low complement(C3 <12 mg%);ANA positivity and positive LE cells.Bacterial culture of pericardial fluid and polymerase chain reaction to Mycobacterium tuberculosis were negative.Pericardial fluid showed no malignant cells on cytological examination.A diagnosis of SLE was offered based on positive clinical and immunologic findings.Pulse intravenous methylprednisolone was given(30 mg/kg/day for 5 days) followed by oral prednisolone(starting at 2 mg/kg/day and tapered thereof).2D-ECHO showed a reduced volume of pericardial fluid with no additional reaccumulation and normal heart function.She remained well for the next 3 years on hydroxychloroquine.However, at age of 17 years she presented with respiratory distress, oral ulcerations, right pleural effusion and recurrence of pericardial effusion and cardiac tamponade.This required pigtail insertion for pericardiocentesis for 8 days.There was laboratory evidence for disease activity(antids-DNA index 2.24;normal <1.1).She responded to oral prednisolone(2 mg/kg/day).A similar episode occurred a year later at 18 years with pericardial and pleural effusion and for this she was given intravenous methylprednisolone pulses for 5 days.In view of recurrent pericarditis, she has been initiated on colchicine(1 mg/day) following which there has been no recurrence of symptoms. Conclusions: Pericardial effusion with cardiac tamponade at presentation of childhood lupus is distinctly unusual and so is the rarity of recurrent pericardial effusion with cardiac tamponade.Our patient continued to have recurrences on follow-up that responded to glucocorticoids. Funding Source(s): None