İmprovement of dilated cardiomyopathy with methylprednisolone in a patient with

multiple fibrosclerosis

Damirbek Osmonov M.D*,Zeki Yuksel Gunaydin M.D*,Göksel Güz M.D ^,Turgay Isik
M.D*.

*Department of Cardiology,Dr.Siyami Ersek Thoracic and Cardiovascular Surgery

Training and Research Hospital,İstanbul,TURKEY
^Department of Cardiology , Faculty of Medicine ,University of
İstanbul,İstanbul,TURKEY
Adress Corresponding to:D. Osmonov M.D. Katip Salih sk 80/1,Kosuyolu
mah,Kadıkoy/İstanbul/Turkey Tel:0090 5058520787
Fax:0090 2163377919
e-mail:damirbeko@yahoo.com
Abstract
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. İn this case,the biopsy specimen of patient demonstrated;
pulmonary hyalinated granuloma or sclerosing mediastinitis.There are few reports of
multiple fibrosclerosis with heart failure.We report a case of retroperitoneal fibrosis with
massive mediastinal involvement extending to pleura and pericardium causing pleuro-
pericardial effusion with dilated cardiomyopathy.Systolic dysfunction improved and
pericardial effusion disappeared with methylprednisolone treatment.
Key words:Multifocal fibrosclerosis,dilated cardiomyopathy,myocarditis, pulmonary
hyalinated granuloma,sclerosing mediastinitis.

1.İntroduction
Retroperitoneal Fibrosis (RPF) is a rare disease with peak incidence in the fifth to
seventh decades of life. Symptoms may be general/nonspecific or localized (due to
replacement or compression of organs). Definitive diagnosis can only be made based on
biopsy findings ,CT scanning or MRI is essential for evaluating the extent of the disease
process. However, some patients require ureteral,intestinal,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
fibrosklerosis.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.
2.Case report
47 year old male who was diagnosed as mediastinal fibrosis 5 month ago with
thoracoscopic biopsy after revealing amfisematous changes and retroperitonal-
paravertebral pleural thickening and pleural effusion on thorax CT.He admitted to
internal medicine with compliants of easy fatigue,vomiting and abdominal pain.
The patient was thin-appearing.On physical examination bilateral bronchovesicular rales
heard on lungs,pleural frotman was absent.Blood pressure was 140/80 mmHg on either
arms,heart rate was 88 beats per minute,s1 and s2 were rhythmyc,s3 and s4 were
absent,2/6 pansystolyc murmur heard from the apex.Epigastric sensitivity observed with
deep palpation and 5 cm of hepatomegali palpated on MIC line.ECG revealed sinus
rhythm and complete RBBB.On chest roentgenogram cardiomegaly with mediastinal
widening observed.There was a marked increase in C-reactive protein and the ESR , but
the serology for connective tissue disease and perinuclear antineutrophil cytoplasmic
antibodies was negative. Creatinin level was elevated to3.7 mg/dl.
On abdominal ultrasound grade II hydronephrosis determined and MR urography
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.After consultation to urology department bilateral
pigtail cathether implanted.
Enlarged all of cardiac chambers,severe LV systolic dysfunction(Ejection
fraction:24%),severe mitral regurgitation,moderate pericardial effusion revealed by
echocardiogram and conventional treatment for heart failure started except for inhibitors
of the renin-angiotensin-aldosterone system.On follow up,creatinin elevated up to 9
mg/dl in conjuction with hyperkalemia,fever,nausea and vomiting.Symptoms relieved
with hemodialysis and administration of intravenous ciprofloxacin,creatinin levels fixed at
4-4.5 mg/dl.
Nephrostomia cannula affixed and urinary output increased up to 6 liters per
day.Creatinin level decreased to 1.6 mg/dl and remained stable. Ureterolysis planned
after 3 moths.We concerned about the malignancy and performed thoracoscopic biopsy
from the most involved site on PET(anterolateral mediastinum on the level of left 6-7 th
intercostal space).Pathological evaluation determined cross-sections of thick walled
vessels,hyalinated connective tissue with lenphoid cells on some areas; pulmonary
hyalinated granuloma or sclerosing mediastinitis.
According to cardiac MRİ,we considered myocarditis as a cuase of dilated
cardiomyopathy (Figure 1),but did not perform biopsy.Patient started on
methylprednisolone 32 mg per day,tapering 4 mg every 2 weeks.After initiation of
corticosteroid teratment,patient feeled himself beter and denied heart failure symptoms.
Control echocardiogram performed two weeks later and ejection fraction increased to
35%,pericardial effusion was minimal.Patient discharged in good clinical situation.On
follow-up as an outpatient manner,1,5 month later on control echocardiogram ejection
fraction was 45%,pericardial effusion has been disappeared (Figure 2).
3.Discussion
Retroperitoneal Fibrosis (RPF) is a rare disease with peak incidence in the fifth to
seventh decades of life.İn this case patient is 47 year old. Its etiology remains to a great
extent unclear; however, its occasional association with autoimmune diseases and its
response to corticosteroids and immunosuppressive therapy suggest it is probably
immunologically mediated
Definitive diagnosis can only be made based on biopsy findings ,CT scanning or MRI is
essential for evaluating the extent of the disease process[1].PET can be used to guide
for biopsy[2].We performed PET and took biopsy from the most involved area and
pathology reported; pulmonary hyalinated granuloma or sclerosing mediastinitis.These
consepts are not estabished terms in the literature. After restoring the function of
involved (hollow) organs, medical therapy with prednisone, immunosuppressive drugs or
tamoxifen is aimed at converting active disease to stable disease.
Multifocal fibrosclerosis is a rare syndrome that is characterized by fibrosis involving
multiple organ systems.However,the presentation of mediastinal-retroperitoneal fibrosis
is rare.İn one series of 491 patients,mediastinal involvement was found in only 3.3% of
idiopathic RPF cases[3]. There were few reported cases of pericardial involvement in
this combined disorder[4]İn the case reported, 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],also the patient reported in this case had these
features.İn our knowledge there is any report of myocarditis and improvement of dilated
cardiomyopathy with methylprednisolone treatment in 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,died with
clinical signs of cardiovascular failure despite pericardiostomy and aggressive
treatment[6].Early administration of steroid therapy for heart failure symptoms due to left
ventricular systolic dysfunction and/or pericardial involvement in patients with multifocal
fibrosclerosis may be beneficial.However, there is no agreement as to the dose and
duration of steroid.
Figure 1:Cardiac MRİ revealing low ejection fraction with dilated cardiac chambers and
diffuse enhancement of myocardium compatible with myocarditis.
Figure 2:Transthoracic echocardiogram showing systolic and diastolic dimensions of left
ventricle.Calculated ejection fraction is 45%.
References:
1)Mulligan SA, Holley HC, Koehler RE, et al. CT and MR imaging in the evaluation of
retroperitoneal fibrosis. J Comput Assist Tomogr 1989; 13:277-281.[Medline]
2)Drieskens O, Blockmans D, Van den Bruel A, Mortelmans L. Riedel’s thyroiditis and
retroperitoneal fibrosis in multifocal fibrosclerosis: positron emission tomographic
findings. Clin Nucl Med 2002; 27:413-415.
3) Koep L, Zuidema GD. The clinical significance of retroperitoneal fibrosis.
Surgery 1977;81:250–257.
4)Klisnick A, Fourcade J, Ruivard M, et al. Combined idiopathic retroperitoneal and
mediastinal fibrosis with pericardial involvement. Clin Nephrol 1999; 52:51-55.[Medline]
5) Amiya E, Ishizaka N, Watanabe A, Endo Y, Itou R, Yoshida S, Nangaku M, Nagai R.
Retroperitoneal fibrosis with periaortic and pericardial involvement.
Circ J. 2005 Jun;69(6):760-2.
PMID: 15914959 [PubMed - indexed for MEDLINE]
6) Omura Y, Yoshioka K, Tsukamoto Y, Maeda I, Morikawa T, Konishi Y, Inoue T, Sato T.
Multifocal fibrosclerosis combined with idiopathic retro-peritoneal and pericardial fibrosis.
Intern Med. 2006;45(7):461-4. Epub 2006 May 1.
PMID: 16679702 [PubMed - indexed for MEDLINE]