Systemic lupus erythematosus (SLE) presenting with cardiac tamponade is a rare cardiac manifestation, Candida pericardial effusion is a very rare entity in clinical settings especially in developing world where tuberculosis is the frequent cause of pericardial effusion, Fungal diseases suspicion arises in the setting of immune suppression like malignancy, diabetes, Steroid treatment and HIV etc, Our case is one such rare event where a treatment naïve young female presented with cardiac tamponade in high disease activity of SLE with superimposed Candida infection.
Systemic lupus erythematosus (SLE) is a prototypical autoimmune disorder that affects multiple organ systems and is prevalent worldwide. (1) Serosal involvement, including pericarditis, is a common feature in SLE and is included in diagnostic criteria established by the American College of Rheumatology (ACR), the Systemic Lupus Collaborating Clinics (SLICC), and the European League Against Rheumatism (EULAR). (2) Pericarditis is one of the most frequent cardiac manifestations in SLE, affecting approximately 9-54% of patients, while cardiac tamponade—a more severe complication—occurs in about 2.5% of cases. (3) Candida pericarditis is an exceptionally rare condition, with limited cases reported in the literature, and is almost uniformly fatal if not treated promptly. (4) The primary risk factors for candidal pericarditis include recent thoracic surgery and an immunocompromised state. (5) This report describes a case of candidal pericarditis presenting with cardiac tamponade in a treatment-naïve SLE patient, highlighting an unusual and severe complication of the disease.
Our patient, a 19-year-old female from rural Uttar Pradesh, presented with fever, joint pain, skin rashes, and weight loss over the past nine months. She also reported swelling in both legs, amenorrhea for six months, and worsening shortness of breath over the past three months, which had become particularly severe in the last week. Notably, she had required two units of packed red blood cells three months prior due to similar complaints. On examination, she exhibited hypotension (blood pressure of 90/60 mmHg) and tachycardia (heart rate of 116 beats per minute). Neck vein engorgement was observed, and an urgent bedside echocardiogram revealed a large pericardial effusion with right ventricular diastolic collapse, indicative of cardiac tamponade (fig 1). An emergency pericardiocentesis was performed, and a pericardial pigtail catheter (fig 2) was inserted. Approximately one litre of pericardial fluid was drained and sent for analysis. The detailed general physical examination of the patient revealed significant findings including hair loss localized to the frontal scalp, painful oral ulcers, and palmo-plantar skin rashes (fig 3). Additionally, she presented with a very low body mass index (15 kg/m²), suggesting severe malnutrition or cachexia. Systemic examination was otherwise unremarkable. Initial laboratory tests showed normocytic normochromic anaemia with a haemoglobin level of 9 g/dL, while total leukocyte and platelet counts were normal. Elevated levels of C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) indicated ongoing inflammation, and elevated ferritin levels were consistent with chronic inflammation. The albumin level was low. Kidney and liver functions were normal. Blood cultures were clear and no growth, Urine pregnancy test and HIV ELISA were negative. Mantoux test was negative. An ultrasound of the thorax revealed bilateral empyema with minimal pleural separation. Despite attempts, ultrasound-guided thoracentesis was unsuccessful. The analysis of the pericardial fluid initially revealed a purulent effusion with markedly low glucose levels and a protein concentration of 3.6 g/dL. The fluid contained 140 cells per microliter, with 90% being polymorphonuclear leukocytes. Given the presence of bilateral empyema and purulent pericarditis, empirical antibiotic therapy with piperacillin-tazobactam and clindamycin was initiated. Further investigations showed that PCR for tuberculosis (TB) and CBNAAT were negative, and the adenosine deaminase (ADA) level was also negative. However, Gram stain identified fungal hyphae (fig 4), and subsequent fungal KOH preparation revealed the presence of Candida tropicalis. On Day 4, reanalysis of the pericardial fluid confirmed an exudative effusion with a predominance of neutrophils, and the fungal KOH test again identified Candida tropicalis, which was sensitive to fluconazole. Consequently, intravenous fluconazole was started to address the fungal infection. The autoimmune panel revealed a strongly positive antinuclear antibody (ANA) test with a homogeneous pattern and an endpoint titre of 1:640. Complement C3 levels were significantly low at 37.10 mg/dL (normal range: 90–180 mg/dL), while complement C4 levels remained normal. Specific antibodies including double-stranded DNA (dsDNA), SmD1, and U1 SnRNP were also strongly positive. Additional antibodies such as those against nucleosomes, histones, Ku, DFS-70, and SSb were detected as well. The patient’s hormonal profile was within normal limits, excluding hormonal imbalances as a contributing factor. An abdominal and pelvic ultrasound revealed mild ascites with normal ovaries and uterus. The secondary amenorrhea was attributed to the chronic autoimmune disease. The patient was ultimately diagnosed with systemic lupus erythematosus (SLE), characterized by high disease activity, as indicated by an EULAR Criteria score exceeding 30 and an SLE Disease Activity Index (DAI) score greater than 15. To manage the condition, she was treated with 1 mg/kg oral steroids and Hydroxychloroquine 5mg/kg/day. After two weeks of steroid therapy and three weeks of antibacterial and antifungal treatment, the patient experienced a significant improvement in her overall well-being and appetite. The pericardial pigtail catheter was removed on the 20th day of admission following three consecutive days of no drainage and a decreasing trend in ESR and CRP levels. She was discharged with a regimen of oral steroids, oral hydroxychloroquine and oral antifungal medication and is currently under follow-up in the rheumatology outpatient department.
Fig 1- Apical 4 chamber view showing large pericardial effusion and parasternal long axis view showing RV Diastolic collapse suggesting Cardiac Tamponade.
Fig 2- Chest X Ray AP view suggesting Gross Cardiomegaly with catheter insitu, and bilateral CP angle blunting is seen which was reconfirmed by USG as Bilateral Empyema.
Fig 3- scalp hair loss non scarring alopecia, flat non tender, reddish macules on palms and soles.
Fig 4- Gram staining of pericardial fluid showing Budding Yeast cells which was later confirmed fungal KOH cultures and speciation identified it to be Candida tropicalis.
Candida is a rare cause of purulent pericarditis, accounting for approximately 1% of cases, with the majority being caused by bacterial infections. (4) Among the Candida species, Candida albicans is the most commonly associated with pericarditis (5), although cases have also been reported with Candida tropicalis, Candida krusei, Candida glabrata, Candida guilliermondii, and Candida parapsilosis. Risk factors for Candida pericarditis include recent thoracic or abdominal surgery, malignancy, and immunosuppression. (6)A flare of SLE refers to worsening disease activity in one or more organ systems, as evidenced by clinical findings and laboratory markers (7) Systemic lupus erythematosus (SLE) can lead to various cardiovascular complications, including myocarditis,
pericarditis, conduction abnormalities, pericardial effusion, and cardiac tamponade. Cardiac tamponade is a particularly rare and severe complication of SLE, affecting about 1% of patients and potentially leading to fatal outcomes if not treated promptly. (8) In the case presented, the patient, who had high disease activity due to SLE, developed cardiac tamponade alongside Candida tropicalis pericarditis. This occurred in an otherwise immunocompetent individual with no history of prior medication or surgery. To our knowledge, this is the first reported instance of such a combination. The presentation of cardiac tamponade in this context may be attributed to an acute exacerbation of SLE and the concurrent fungal infection. Invasive fungal infections (IFIs) can occasionally affect SLE patients, with opportunistic pathogens like Aspergillus, Cryptococcus, and Candida being common culprits. These infections can involve multiple systems, including the urinary tract, lungs, spleen, central nervous system, pharynx, oesophagus, or present as fungemia. Several factors can increase the risk of IFI in SLE patients, including high doses of corticosteroids, diabetes, elevated C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR), and high Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) scores. A study by Fan et al. of 1,500 SLE patients revealed that those on higher steroid doses, with concurrent diabetes, and elevated inflammatory markers, were at a higher risk for invasive fungal infections. (9), (10)
Invasive fungal infection is potentially life threatening event in SLE patients, cardiac tamponade precipitated by invasive fungal infection in a treatment naïve SLE patient with high SLE disease activity, is a rare manifestion and re-iterates the fact of occurrence of invasive fungal disease in such patients even prior to imuuno supresive therapies.
Download PDF
Download PDF
Download PDF
Download PDF
+91 9063922599
+91 9063922599
editor@healthcare-bulletin.co.uk
Download XML
