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Features of Nephrogenic Systemic Fibrosis on Radiology Exam

Features of Nephrogenic Systemic Fibrosis on Radiology Exam

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Radiological findings on patients with a confirmed diagnosis of nephrogenic systemic fibrosis.
Radiological findings on patients with a confirmed diagnosis of nephrogenic systemic fibrosis.

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AJR:193, July 2009 61
gen bundles seen in the dermis, and otenincreased dermal deposits o mucin.These criteria have been expanded in a re-cent review [32].Because NSF primarily involves the skin,little attention has been paid to its imagingcharacteristics [33]. We have seen the imag-ing studies o 26 biopsy-conrmed cases o NSF at our institution. This article illustratesthe spectrum o imaging ndings in theseNSF patients with photographic and histo-pathologic correlation.
Histopathology
Deep punch biopsy specimens o NSF le-sions show, in varying proportions, spindlecell prolieration, thickened collagen bun-dles, and mucin deposition (Fig. 1) in a pro-cess believed to be mediated by circulatingbrocytes inappropriately stimulated by gad-olinium [34]. Although NSF primarily a-ects the skin, involvement o skeletal mus-cle, heart, and lungs has also been reported[23, 32, 35]. The histologic eatures o NSFcan be very similar in appearance to otherdermatologic conditions, including sclero-myxedema, scleroderma and deep morphea,lipodermatosclerosis, eosinophilic asciitis(Shulman syndrome), and chronic grat-ver-sus-host disease [32]. Thus, diagnosing NSFrequires a combination o characteristic his-tologic and clinical ndings.
Clinical Manifestations
All NSF patients have signicant renalimpairment, with an estimated GFR o < 30
Features of Nephrogenic SystemicFibrosis on Radiology Examinations
Michael F. Morris
1
Yang ZhangHonglei ZhangJoan C. ProwdaDavid N. SilversRashid A. FawwazMartin R. Prince
Morris MF, Zhang Y, Zhang H, et al.
1
All authors: Department o Radiology and Departmento Dermatology and Dermatopathology at Columbia andCornell Universities, 416 E 55th St., New York, NY 10022.Address correspondence to M. R. Prince(map2008@med.cornell.edu).
Gastrointestinal Imaging • Pictorial Essay
AJR 
2009; 193:61–690361–803X/09/1931–61© American Roentgen Ray Society
N
ephrogenic systemic brosis (NSF)is a rare disease seen in patientswith severe renal impairment thathas garnered increased interestamong radiologists because o reports o its as-sociation with gadolinium-based contrast agents(GBCAs) [1–20]. Many case series have report-ed that high doses o GBCA and possibly o lin-ear nonionic GBCA contribute to an increasedrisk o NSF [16, 17, 21–25]. These ndingshave led to recommendations or radiologiststo avoid the use o GBCA or reduce the doseo GBCA in patients with an estimated glom-erular ltration rate (GFR) o less than 30 mL/ min [26]. It is also recommended that dialysispatients undergo dialysis immediately aterGBCA injection to urther minimize the risk o NSF when GBCA is essential [27].NSF was originally reported by Cow-per et al. [28] to have rst occurred in 1997.NSF aects male and emale patients equal-ly and has been reported to occur in patientso all ages including children as young as 8years old [29]. Although the cause o NSF re-mains unknown, most patients have a historyo gadolinium exposure and gadolinium hasbeen detected in NSF skin lesions, as report-ed by several authors [9, 30].Cowper [31] has established the ollowingdiagnostic criteria or NSF:…large areas o hardened skin withslightly raised plaques, papules, or con-fuent papules; with or without pigmen-tary alteration and/or with biopsies show-ing increased numbers o broblasts,alteration o the normal pattern o colla-
Keywords:
CT, gadolinium, mammography, MRI,nephrogenic systemic brosis, PET, scintigraphy,ultrasoundDOI:10.2214/AJR.08.1352Received June 5, 2008; accepted ater revisionDecember 25, 2008.M. R. Prince has patent agreements with the ollowingcompanies, which manuacture gadolinium-basedcontrast agents: GE Healthcare, Bayer HealthCare,Mallinckrodt Imaging, Bracco Laboratories, and Epix.
    F    O    C    U    S    O    N   :
OBJECTIVE.
The objective o this article is to illustrate the spectrum o imaging nd-ings with photographic and histopathologic correlation in patients with biopsy-proven neph-rogenic systemic brosis (NSF).
CONCLUSION.
Features o NSF may be evident on the patient’s skin as well as on rou-tine imaging studies, although these imaging ndings are nonspecic and are more likely tooccur with other diseases.
Morris et al.Imaging Features o NSFGastrointestinal ImagingPictorial Essay
 
62 AJR:193, July 2009
Morris et al.
mL/min (stage IV or V kidney disease), dial-ysis-dependent end-stage renal disease, oracute renal ailure. Patients oten initiallypresent with nonspecic complaints—includ-ing joint pain, extremity swelling, and sti-ness—that in turn may lead to multiple imag-ing procedures. On physical examination, thecutaneous maniestations o NSF commonlyinvolve the extremities and trunk beginningdistally and spreading proximally while spar-ing the ace. Patients with mild disease canpresent with a variety o nonspecic ndingsranging rom erythema, papules, and plaques(Fig. 2) to skin dimpling and peau d’orange(Fig. 3). Patients may develop contracturesthat reduce mobility (Fig. 4). Although mostcases o NSF are sel-limiting and some re-gress spontaneously, an estimated 5% have arapidly progressive ulminate course [23].Unlike allergic reactions that happen with-in minutes o injection and are independento contrast agent dose, the skin changes o NSF typically are delayed, occurring rom 2weeks to 2 months ater high-dose GBCA in- jection. NSF is rare with standard 0.1 mmol/ kg doses o gadolinium [27]. There may bean initial prodrome o muscle pain, ever,weakness, swelling, redness, pruritus, or painin the limbs sometimes with muscle weak-ness, edema, or erythema and occasionallywith palpable warmth o the involved extrem-ities; there may be forid scleral telangiec-tasia resembling conjunctivitis [36]. Clinicalseverity is related to both the extent o thebrosing dermopathy and the degree o sys-temic involvement. Muscle involvement tendsto be underlying the cutaneous lesions withbrotic bands in the subcutaneous tissuestethering the skin to the underlying ascia. Insome patients the NSF lesions resolve withrestoration o normal renal unction ater re-nal transplantation or resolution o acute re-nal ailure. In many cases the lesions persist,although a recent report shows promising re-sults or treating patients with NSF usingimatinib mesylate [37].
Radiography
The conventional radiographic ndings o NSF are nonspecic, and abnormalities onradiographs are rare and usually are second-ary to the sequelae o joint contracture andimmobility. Flexion deormities and disuseosteopenia rom contractures occur mostcommonly in the eet (Fig. 4), knees, hands,and elbows. In 140 NSF patients described indetail in case reports, limited range o mo-tion or joint contractions were noted clinical-ly in 106 (58%) [38]. We saw joint contrac-tures on radiographs in two o our 26 patients,but the contractures in one patient were dueto juvenile rheumatoid arthritis and had beenpresent beore NSF developed. Note that thedierential diagnosis or joint contracturesincludes arthritides and immobilization (e.g.,due to trauma, burns, nerve injury, or stroke);the likelihood that a joint contracture signi-es NSF is negligible.Although microscopic dermal calcica-tions are common in NSF specimens andCowper and colleagues have described skincalcications on radiographs, these calcica-tions are rare in NSF cases [39]. We oundcutaneous calcications on an imaging studyin only one o our 26 biopsy-positive NSF pa-tients. In that patient, skin calcications onabdominopelvic CT were thought to be morelikely related to the patient’s other diseasesincluding juvenile rheumatoid arthritis, dial-ysis-dependent end-stage renal ailure, andsystemic lupus erythematosus. Vascular cal-cications visible on radiographs are usuallyrelated to diabetes or renal ailure.
Mammography
Three o the six emale NSF patients whounderwent mammography at our hospital hadskin thickening and increased subcutaneouslinear markings (Fig. 5) corresponding toNSF lesions. Although the breast skin thick-ening in these patients was readily identiedas part o the NSF process occurring overthe legs, arms, and chest, skin thickening onmammography is much more likely to be re-lated to infammatory breast cancer, cellulitis,scarring, or venous or lymphatic obstruction.
Ultrasound
Duplex ultrasound may be perormed toassess or extremity deep venous thrombo-sis (DVT) in patients with NSF because o the common complaints o extremity painand swelling. Breast ultrasound may be per-ormed in emale patients with NSF whohave mammographic abnormalities, and itmay show skin thickening and subcutane-ous edemalike infammatory changes. Notethat these ndings are nonspecic; are muchmore likely to result rom DVT, infamma-tion, or cellulitis; and would not be expectedto elicit the diagnosis o NSF.
CT
The most common CT study perormedin patients with NSF is o the abdomen andpelvis; however, occasionally CT o an ex-tremity is perormed during a workup or ex-tremity pain. Depending on the severity o disease, CT may show varying degrees o skin thickening and inltration o the subcu-taneous and sot tissues (Figs. 6 and 7). Re-ormations in coronal and sagittal planes andalong the axis o the bones may acilitate as-sessment o dermal inltration. In particu-lar, bands o collagen in the subcutaneous attether the skin to underlying sot tissues [40].Systemic involvement o the internal organsis dicult to identiy on CT and thus hasbeen reported only at autopsy or on surgicaland biopsy specimens. The dierential diag-nosis or skin thickening and inltration o the subcutaneous at on CT includes edema,cellulitis, ascitis and panniculitis, sclero-derma and sclerodermalike conditions (e.g.,morphea), cutaneous T-cell lymphoma, andMerkel cell carcinoma.
MRI
The spectrum o abnormal MRI ndingsin patients with NSF can be similar to thatseen on CT. Subtle areas o edema are seen onSTIR or T2-weighted at-saturated images inpatients with mild clinical maniestations o NSF, whereas patients with more severe in-volvement show skin thickening and diuseinfammatory changes throughout the sottissues (Figs. 8–10). Although gadolinium iscontraindicated in patients with severe renalimpairment, enhancement o the subcutane-ous tissues and skeletal muscle may be seenon T1-weighted at-saturated images whenGBCA is administered [24]. These nonspe-cic ndings are seen in many infammato-ry, neoplastic, and traumatic conditions andshould not raise suspicion or NSF.
Nuclear Medicine
Extraosseous accumulation o 
99m
Tc-hy-droxydiphosphonate (HDP) can be seenin NSF patients undergoing bone scintig-raphy (Fig. 11) and was previously report-ed [32, 41, 42]. The dierential diagnosisor diuse extremity sot-tissue uptake onbone scans includes myositis ossicans andheterotopic calcications; dermatomyositis;rhabdomyolysis; polymyositis; metastaticcalcications; and technical actors, includ-ing contamination and tourniquet eects.There is a case report describing FDG up-take in NSF [41]. We ound only one NSFpatient with FDG uptake on PET and that pa-tient had cutaneous T-cell lymphoma, whichwas more likely to be the reason or FDG ac-tivity in the skin.
 
AJR:193, July 2009 63
Imaging Features of NSFConclusion
NSF has a variable appearance on routineimaging studies. Oten there are no abnor-mal imaging ndings. However, convention-al radiographs can show joint contractures,skin thickening, and possibly cutaneous cal-cinosis; ultrasound may show thickening andedema o the cutis, particularly in the breast;CT may show skin thickening and inltra-tion o subcutaneous tissues; MRI may showincreased signal on fuid-sensitive sequenc-es in the skin, subcutaneous tissues, and ex-tremity musculature; and bone scintigraphymay show diuse sot-tissue uptake in theextremities. Studies o the head and anteri-or neck ail to show changes related to NSFbecause the disease spares that region. Allthese imaging ndings are nonspecic andcannot be used to diagnose NSF using thecurrently accepted diagnostic criteria.
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