retroperitoneal fibrosis ( rpf ) is a rare disease with peak incidence in the fifth to seventh decades of life .
the symptoms of rpf may be general / nonspecific or localized ( due to replacement or compression of organs ) . yet
, definitive diagnosis can only be made based on biopsy findings . besides , magnetic resonance imaging ( mri ) is essential for evaluating the extent of the disease process ( 1 ) .
however , some patients require ureteral , intestinal , or paravascular surgery due to obstruction in spite of medical treatment
. there are few reports of retroperitoneal and mediastinal fibrosis with pleural and pericardial involvement described as multifocal fibrosclerosis ( 2 ) . here , we present a case of retroperitoneal fibrosis with massive mediastinal involvement extending to pleura and pericardium causing pleural , pericardial effusion , and heart failure , with improvement after methylprednisolone administration .
the case was a 47-year - old man who was diagnosed with mediastinal fibrosis 5 months ago with thoracoscopic biopsy after presenting emphysematous changes , retroperitonal - paravertebral pleural thickening , and pleural effusion on thorax ct .
he was admitted to internal medicine with complaints of easy fatigue , vomiting , and abdominal pain .
his blood pressure was 140/80 mmhg on either arms , his heart rate was 88 beats per minute , s1 and s2 were rhythmyc , s3 and s4 were absent , and 2/6 pansystolyc murmur was heard from the apex .
reticulo - nodular consolidation areas were also detected in the left side of the lung .
there was a marked increase in c - reactive protein and the erythrocyte sedimentation rate ( esr ) , but the serology for connective tissue disease and perinuclear antineutrophil cytoplasmic antibodies was negative .
nevertheless , creatinine level was elevated to 3.7 mg / dl . using abdominal ultrasound , grade
ii hydronephrosis was determined and mr urography was performed ; soft tissue from basal pole of both kidneys extending to minor pelvis , on paraaortocaval area and on iliac chains , was like retroperitoneal fibrosis surrounding both ureters at iliac crossing level .
enlarged cardiac chambers , severe left ventricular ( lv ) systolic dysfunction ( ejection fraction : 24% ) , severe mitral regurgitation , and moderate pericardial effusion were revealed by echocardiogram and conventional treatment for heart failure was started , except for inhibitors of the renin- angiotensin - aldosterone system . during the follow up ,
creatinine level was elevated up to 9 mg / dl in conjunction with hyperkalemia , fever , nausea , and vomiting . however , the symptoms were relieved by hemodialysis and administration of intravenous ciprofloxacin .
creatinine level was also fixed at 4 - 4.5 mg / dl . additionally , nephrostomia cannula was affixed and urinary output was increased up to 6 liters per day
. then , the creatinine level decreased to 1.6 mg / dl and remained stable thereafter .
we were concerned about the malignancy and performed thoracoscopic biopsy from the most involved site on positron emission tomography ( pet ) ( anterolateral mediastinum on the level of left 6 - 7 the intercostal space ) .
pathological evaluations revealed cross - sections of thick walled vessels , hyalinated connective tissue with lenphoid cells on some areas , pulmonary hyalinated granuloma , and sclerosing mediastinitis . according to cardiac mri
, we considered myocarditis as a cause of dilated cardiomyopathy ( figure 1 ) , but did not perform biopsy .
initially , the patient received methylprednisolone 32 mg per day , tapering 4 mg every 2 weeks .
after initiation of corticosteroid treatment , the patient felt better and denied heart failure symptoms .
1.5 months later , the control echocardiogram showed ejection fraction to be 45% ( figure 2 ) .
rpf is a rare disease with peak incidence in the fifth to seventh decades of life . in the present study patient who was a 47-year - old man ,
however , its occasional association with autoimmune diseases and its response to corticosteroids and immunosuppressive therapy suggested that it was probably immunologically mediated .
mri is also essential for evaluating the extent of the disease process ( 1 ) . besides
, pet can be used to guide biopsy ( 3 ) . in the present study ,
these concepts are no established terms in the literature . after restoring the function of the involved ( hollow ) organs , medical therapy with prednisone , immunosuppressive drugs , or tamoxifen
multifocal fibrosclerosis is a rare syndrome that is characterized by fibrosis involving multiple organ systems .
however , presentation of mediastinal - retroperitoneal fibrosis is rare . in one series of 491 patients ,
mediastinal involvement was found in only 3.3% of the idiopathic rpf cases ( 2 ) . nonetheless , there were few reported cases of pericardial involvement in this combined disorder ( 4 ) . in the case
reported here , the patient had retroperitoneal fibrosis and massive mediastinal fibrosis with pleuro - pericardial involvement
. there were some reports of multifocal fibrosclerosis with dilated cardiomyopathy as case reports in the literature ( 5 ) .
to the best of our knowledge , no report of myocarditis and improvement of dilated cardiomyopathy with methylprednisolone treatment is available in the patients with multifocal fibrosclerosis .
omura et al . in 2006 reported a case of multifocal fibrosclerosis combined with idiopathic retro- peritoneal and pericardial fibrosis who had massive pericardial effusion and died with clinical signs of cardiovascular failure despite pericardiostomy and aggressive treatment ( 6 ) .
early administration of steroid therapy may be beneficial for heart failure symptoms due to lv systolic dysfunction and/or pericardial involvement in the patients with multifocal fibrosclerosis
. however , there is no agreement on the dose and duration of steroid therapy .
 multifocal fibrosclerosis is a rare syndrome of unknown cause that is characterized by fibrosis involving multiple organ systems . 
 definitive diagnosis can only be made based on biopsy findings . in this case , the biopsy specimen of the patient demonstrates pulmonary hyalinated granuloma or sclerosing mediastinitis . 
 there are few reports of multiple fibrosclerosis with heart failure . here , we reported a case of retroperitoneal fibrosis with massive mediastinal involvement extending to pleura and pericardium causing pleuro- pericardial effusion with dilated cardiomyopathy . 
 systolic dysfunction was improved and pericardial effusion disappeared with methylprednisolone treatment . 
1. Introduction
2. Case Presentation
3. Discussion