Background

Pericarditis has a hospitalization rate of 3.32 and an incidence of 27.7 per 100,000 person-years and accounts for only about 0.2% of all cardiac-related hospitalizations [1]. Triggers may include infections (viral, bacterial, fungal, parasitic) as well as noninfectious triggers (autoimmune diseases, neoplasms, metabolic disorders, and iatrogenic and traumatic causes) [2]. In very rare cases, drugs, especially chemotherapeutic agents and rarely penicillins, can also lead to the development of pericarditis.

To diagnose pericarditis, more than two of the following parameters must be present: chest pain (improvement by sitting upright, leaning forward), pericardial rubbing, typical ECG changes, new-onset or worsening pericardial effusion, elevation of infectious parameters, evidence of pericardial inflammation by imaging. Prognostically, fever, recurrent hemodynamically effective pericardial effusions, and nonresponse to NSAID therapy [3] are associated with prolonged hospital stays. Treatment is predominantly by anti-inflammatory drugs (NSAIDs, colchicine, and glucocorticoids) as well as treatment of the underlying disease [4]. However, the data regarding drug-induced pericarditis are sparse [5].

Case report

A 64-year-old female hypertensive patient with increasing dyspnea and unspecific thoracic pain had had rheumatoid arthritis for 20 years, which was treated with methotrexate, leflunomide, and abatacept as mono- and combination therapy. Due to continued disease activity, the patient received a combination therapy of methotrexate with etanercept 6 months ago. Bilateral pneumonia with Staphylococcus aureus bacteremia was successfully treated. The patient had had recurrent pericardial and pleural effusions for years, which were attributed to rheumatoid arthritis activity. For treatment of the recurrent effusions, the patient received glucocorticoids as well as colchicine in addition to the basic rheumatoid arthritis therapy. On admission, she complained of malaise, vomiting, and dysphagia; blood pressure was 85/55 mm Hg, she was normofrequent, inflammatory parameters were elevated. On TTE and CT, there was a 3-cm non-floating structure in the entire circumference of the pericardium, thickened to 3 mm (Fig. 1). The indication for partial pericardiectomy was given because of hemodynamic impairment. Six weeks earlier, a subxiphoid pericardiotomy had been performed with nonspecific histology and negative cytology. After incision of the pericardium, 250 ml of a brown-reddish fluid drained, with brown crumbly necrotic masses visible underneath (Fig. 2). The pericardium was resected except for the dorsal portions. After pericardiectomy, the patient remained free of cardiac symptoms for 12 months (to date). Immunosuppressive therapy was significantly reduced, and the patient last received only immunomodulatory and anti-inflammatory therapy with hydroxychloroquine and low-dose methylprednisolone. Since bilateral pneumonia and subsequently osteomyelitis occurred during this therapy, an immunodeficiency was suspected. This was confirmed and a substitution therapy with immunoglobulins was initiated.

Fig. 1
figure 1

CT (a) morphological as well as (b) ultrasound-targeted visualization of circumferential extension

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Fig. 2
figure 2

Opening of the pericardium (a) with discharge of 250 ml of a dark brown, serous fluid. (b) Underneath, a solid, partly necrotic mass was revealed

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Diagnosis

Histopathologic findings revealed vasculitis-related chronic fibrinous pericarditis with vasculitic changes in the small vessels without evidence of malignancy. Cytologically, there were no pathologic findings; subclinical infection with Staphylococcus aureus was detectable by PCR analysis. However, microbiologically, no evidence of florid infection was found.

Discussion

Based on the fact that tumor necrosis factor blockers can induce vasculitis and lupus-like polyserositis, etanercept might have been responsible for the exacerbation of pericarditis. [6, 7]. The underlying rheumatoid arthritis could also be considered as a trigger of pericarditis since the incidence of pericarditis is reported in the literature as up to 27% [8]. Based on the detection of Staphylococcus aureus DNA in the pericardium, the S. aureus bacteremia that took place, and the small vessel vasculitis that was detected, the exacerbation of pericarditis was attributed to secondary vasculitis after an infection with S. aureus, whereas the tendency to infection due to humoral immunodeficiency after years of immunosuppressive therapy including continuous glucocorticoid application has to be discussed as a trigger.