Constrictive pericarditis as the presenting manifestation of systemic sclerosis

Internal organ involvement can manifest prior to the onset of skin changes in SSc. Sir, We present a 62-year-old male, diagnosed with SSc 16 years after the disease was first manifest by chronic constrictive pericarditis (CP). The patient presented with a 1-year history of lethargy, orthopnoea and exertional dyspnoea at age 46 years. Physical examination revealed signs of cardiac failure. Laboratory tests were within normal limits. No autoimmune serology was performed. Coronary angiogram revealed no coronary artery disease. Echocardiography showed thickening of the pericardium with constriction of the right ventricle. Cardiac MRI demonstrated diffuse pericardial thickening. The patient proceeded to pericardiectomy. Histopathological analysis revealed dense fibrotic thickening of the pericardium with focal nodular lymphoplasmacytic infiltration around vessels and the pericardium (Fig. 1A). Immunohistochemistry showed the majority of lymphocytes were small B-cells (CD20+) with smaller numbers of small T cells (CD3+). Retrospectively, immunohistochemistry for IgG4 was performed and up to 27 IgG4+ plasma cells per high power field were noted, with an IgG4+/IgG+ ratio of 30% (Fig. 1B). The ratio of IgG4+/IgG+ plasma cells was below the required cut off of ≥40% for a diagnosis of IgG4-related disease. In light of this, the pericarditis was not felt to be caused by IgG4-related disease. There was no evidence of malignancy or granulomas and no bacterial or mycobacterial organisms were cultured. The patient made a full recovery. Histopathology of pericardium (A) Low-power magnification of the pericardium, which shows fibrous thickening and nodular lymphplasmacytic infiltrates (haematoxylin and eosin); (B) high-power maginification of pericardium showing nodular infiltrates of lymphocytes and plasma cells (haematoxylin and eosin). At age 61 years, the patient presented with a 12-month history of RP, progressive skin thickening, reflux and dyspnoea. On examination, there was evidence of sclerodactyly, digital ulceration and skin thickening to the mid-forearms, with truncal sparing. Bibasal fine inspiratory crepitations were audible. Autoimmune serology revealed positive ANA in nucleolar (titre 1:640) and homogeneous (titre >1:1280) patterns, positive anti-topoisomerase I and anti-Ro52 antibodies. ECG revealed atrial fibrillation. Echocardiography showed left atrial enlargement and no features of constriction, ventricular failure or pulmonary hypertension. Respiratory function tests revealed reduced diffusing capacity of the lung at 53% predicted and normal spirometry. CT of the chest showed a small volume of interstitial lung disease. Coronary angiogram and right and left heart catheterization revealed severe left anterior descending artery stenosis, elevated pulmonary artery pressure (29 mmHg), elevated pulmonary capillary wedge pressure (22 mmHg) and normal pulmonary vascular resistance (1.4 Wood units). A permanent pacemaker was required for management of atrial fibrillation after unsuccessful direct-current cardioversion, and rivaroxaban was commenced. A proton pump inhibitor and calcium-channel blocker were started for treatment of SSc symptoms. This case illustrates that SSc can have a highly variable presentation and autoimmune disease should be considered in the diagnostic work-up of patients with unexplained CP. Typical features of SSc are the presence of RP, fingertip changes and skin thickening in association with positive ANA and internal organ fibrosis. However, the presentation of SSc can be highly varied with internal organ manifestations developing prior to the onset of skin thickening [1]. To our knowledge, this is the first case reported of SSc presenting with CP due to fibrotic pericarditis. CP of any aetiology is an uncommon disorder and in developed countries, it is most commonly considered an idiopathic disorder or the result of previous cardiac surgery, acute pericarditis or radiotherapy [2]. Clinically, symptomatic pericardial disease is reported in 1.9–35% of SSc patients, and generally presents either as acute pericarditis or chronic pericardial effusion [3]. The prevalence of SSc-associated CP is unknown. Determining the aetiology of CP in the absence of any other signs of systemic disease can be challenging as the histopathological changes of the pericardium associated with SSc are fibrous or fibrinous pericarditis with or without chronic inflammatory cells, similar to other causes of CP [2, 4, 5]. Calcification of the pericardium has only rarely been reported in patients with SSc [2, 6]. Autopsy studies have demonstrated that 11–24% of SSc patients have evidence of fibrous pericarditis and up to 28% of patients have evidence of pericardial adhesions [4, 5]. However, the relationship between clinical symptoms and histopathological findings is not well understood as these findings are often asymptomatic; in one series, only one of five patients with evidence of fibrous pericarditis at autopsy had clinical symptoms of CP [5]. The optimal treatment of CP is unclear. The role, if any, of immunosuppression is unknown. In acute CP of any aetiology, evidence of pericardial inflammation on imaging indicates a role for anti-inflammatory therapy [2]. Case reports have suggested using CS either as monotherapy or in combination with other immunosuppression in cases of SSc. However, almost half of reported cases of SSc-associated pericarditis are not pharmacologically treated [7]. Furthermore, the use of CS in SSc is controversial, as steroid use has been linked to an increased risk of scleroderma renal crisis [8]. In conclusion, we present a case of SSc presenting with CP, a rare manifestation of SSc. Underlying autoimmune disease should be considered in all cases of unexplained CP. Funding: This work was supported by Musculoskeletal Australia (PhD scholarship to L.R.), an Australian Government Research Training Program Scholarship (to L.R.) and a National Health and Medical Research Council of Australia Fellowship (APP 1126370 to M.N.). Disclosure statement: The authors have declared no conflicts of interest.