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Erdheim-Chester DiseaseA Case Study and Literature Review Diagnosis and treatment of patients who present with respiratory compromise are challenging. What happens when these patients do not respond to your intervention, and their condition declines rapidly? Having a variety of differential diagnoses is key. An addition to your differential list can include a rare disorder of nonYLangerhans cellshistiocytosis also known as Erdheim-Chester disease. This disease often presents as an interstitial lung disease that fails many different treatment modalities. A full understanding of how this disease process works is still being investigated. Provided are a literature review and case study for better understanding of this disease.
Keywords: Erdheim-Chester disease, Histiocytosis, Idiopathic pulmonary [DIMENS CRIT CARE NURS. 2011;30(4):184/189] Erdheim and William Chester first discovered Erdheim- structive sleep apnea noticed increasing dyspnea on Chester disease in 1930. It is a disease process with only exertion and orthopnea, which has progressively wors- 250 reported cases.1 It typically affects middle-aged and ened over the past 3 years. She also experienced a 30-lb elderly men.2 It is a progressive disease with multisystem weight gain. She was initially seen by a cardiologist for involvement. Mortality is 57% typically from respiratory signs and symptoms of congestive heart failure (CHF). A distress, and because of its rarity and wide spectrum of transthoracic echocardiogram was done that showed clinical manifestations, it is difficult to diagnose and treat.
normal findings. She was treated for fluid overload and According to literature, pulmonary involvement has the was administered diuretics. Recently, she was hospitalized worst prognosis because 35% of patients with a diagnosis for a ruptured ectopic pregnancy that was complicated by of Erdheim-Chester disease have pulmonary involvement.3 acute kidney injury, hemolytic anemia requiring steroids, Diagnosis of Erdheim-Chester is confirmed by histopa- left perinephritic abscess, and recurrent pancreatitis status thology (nonYLangerhans histiocytosis) and radiological post pancreatoduodenostomy and splenectomy. She was studies of the long bones.4 Oftentimes, Erdheim-Chester discharged on a 6-week steroid taper for the hemolytic disease is misdiagnosed as interstitial lung disease or other anemia and required home oxygen. She stated that her pulmonary disorders. When evaluating patients with pos- breathing was the best it has ever been while she was on the sible idiopathic pulmonary fibrosis, Erdheim-Chester steroids; however, once the steroids were completed, her should be one of your differential diagnoses.
breathing worsened. One month later, she presented to theemergency department with severe dyspnea and bilateral lower-extremity edema. Her arterial blood gas revealed a A 34-year-old woman with a medical history of hyper- hypoxic respiratory acidosis, and chest x-ray revealed tension, type 2 diabetes mellitus, hyperlipidemia, and ob- bilateral infiltrates. She was admitted to the medical Copyright @ 2011 Lippincott Wil iams & Wilkins. Unauthorized reproduction of this article is prohibited.
S-100 protein, suggestive of Erdheim-Chester disease. The disease was then confirmed by long-bone x-rays, which showed diffuse sclerosis throughout the metaphyses and diaphyses in the long bones of the lower extremities. Thepatient was unable to wean from mechanical ventilation after 16 days. At this time, she was started on pulse dose steroids and was extubated 2 days later. Her hospital course was again complicated by multiple readmissions to the MICU requiring 2 intubations and mechanical ven-tilation for respiratory failure. After multiple steroid adjustments, she stated that her lungs felt ‘‘fuller and can now breathe easier.’’ She also developed leukocytosis from a suspected central line infection, and once her infection clears, she will be administered interferon " and cyclo-sporins for suggested management of Erdheim-Chester Results from the lung biopsy were
positive for the S-100 protein
suggestive of Erdheim-Chester disease.
With current information provided, Erdheim-Chester disease is known to be a rare nonYLangerhans cellhistiocytosis with progressive multisystem involvement.1 NonYLangerhans cell histiocytosis refers to a group of conditions called histiocytosis that are caused by an 2, partial pressure of arterial carbon dioxide; pH, hydrogen ion concentration; PO2, partial pressure of arterial oxygen.
overgrowth of cells called histiocytes. Histiocytes can bedivided into 2 groups based on their characteristics. The intensive care unit (MICU), where she was intubated for first group is a monocyte-macrophage group that in- respiratory failure and hypoxia. Her laboratory and cludes monocytes, macrophages, and Kupffer cells.5 The radiographic findings on admission are shown in Table 1.
second group is the Langerhans cellYderived group and is Her chest x-ray showed bilateral edema and/or in- the histiocytic cell type that is responsible for Langerhans filtrates with layering right greater than left pleural cell histiocytosis. NonYLangerhans cell histiocytosis is effusions, whereas her chest computed tomography (CT) split into 2 groups, classes IIa and IIb. Class IIa involves scan of the pulmonary system revealed smooth but diffuse dermal dendritic cells and includes dermatofibroma, bilateral interlobular septal thickening and bilateral trace xanthogranuloma, reticulocytosis, and Erdheim-Chester pleural effusions. Cardiovascular system showed multi- disease. Class IIb involves cells other than Langerhans chambered cardiomegaly with a hyperdense septum and cells and dermal dendrocytes. This class includes heredi- tary and acquired diseases such as familial hemophago- During her stay in the MICU, she had acute kidney cytic lymphohistiocytosis, familial sea-blue histiocytosis, injury with 80% right renal artery stenosis, in addition to and hereditary progressive mucinous histiocytosis.6 The her left perinephritic abscess and multilobe pneumonia.
etiology of this disease process is still unknown. The dis- After an appropriate course of antibiotic treatment for ease has been described as a rare focal or systemic in- the multilobe pneumonia without ventilation improve- filtrative disorder resulting from xanthogranulomatous ment, a pulmonary artery catheter was placed to differenti- infiltration of multiple tissue and organs.2 In most of the ate cardiogenic versus pulmonary etiology for appropriate cases, the bone involvement is constant and includes sym- volume status management. Pulmonary artery wedge pres- metric osteosclerosis of the diaphyses and metaphyses of sures were normal, and a lung biopsy was conducted the long bones, especially of the lower extremities.5 The at this time. Results from the lung biopsy were positive for differentiation between Erdheim-Chester disease and Copyright @ 2011 Lippincott Wil iams & Wilkins. Unauthorized reproduction of this article is prohibited.
Langerhans cell histiocytosis is in its histopathology.
Erdheim-Chester differs in its age distribution and im- Retroperitoneal involvement is from the infiltration of fat munochemical and radiological characteristics.7 and surrounding structures by histiocytes.7 It may occurin isolation of a single organ or present as a widespread disease with multiple organ involvement. Histiocytic in- There are several clinical manifestations of Erdheim- filtrates may impair retroperitoneal organs including the Chester disease, which makes it difficult to diagnose and kidney, pancreas, or adrenal gland. Also, perivascular in- treat (Table 2). Extraskeletal manifestations may affect filtrates may cause some stenosis of renal arteries as well the lungs, pericardium, aorta, retroperitoneum, skin, as some biliary obstruction from pancreatic head involve- and ocular orbits. The most common manifestations in- ment. In some cases, renal involvement is included by the clude skeletal, neurological, orbital, retroperitoneal, and direct invasion of the renal sinus or by distal ureter obstruction.10 One study done by Haroche and colleagues11studied 22 patients with Erdheim-Chester disease and sug- gested that a possible cause of morphological changes in Knee pain and leg pain are the most common symptoms adrenal size and infiltration could be related from Erdheim- because the disease affects mainly the lower extremities.7,8 The findings on plain long-bone x-rays show osteoscle-rosis of the diaphyses and metaphyses with sparing of the epiphyses.7 Also on bone scintigraphy is bilateral sym- The most common presentation with pulmonary involve- metric uptake of bone seeking radiopharmaceutical with- ment is progressive dyspnea as the case scenario patient in the metadiaphyses of the skeleton.7 The patient in the presented.7 Approximately 20% of patients present with case study never complained of pain.
pulmonary involvement, which has a poor prognosis.12A review of literature by Allen and colleagues had pul- monary involvement.13 Eighteen of those patients had an The most common neurological manifestations are dia- overall survival rate of 66%. Eleven of those patients betes insipidus and ataxia of gait. These symptoms arise died of their diseases, 3-16 years of life span from time of from lesions found on magnetic resonance imaging, which diagnosis.13 Patients with pulmonary involvement present are typically seen on the cerebellum and pons. Magnetic with signs and symptoms of interstitial lung disease, mostly resonance imaging findings are often confused with mul- cough and shortness of breath.14 Patients with cardiac tiple sclerosis by showing a demyelinating process.7 involvement in addition to the primary pulmonary in-volvement have a worse prognostic outcome. Pul- monary cases were reviewed by Veyssier-Belot and Orbital involvement in Erdheim-Chester disease usually colleagues,4 which showed that many of the patients’ mani- involves middle-aged and elderly patients, rarely children.
festations included nonspecific symptoms such as dyspnea The classic signs of orbital involvement are bilateral and cough, which may be from underlying pulmonary, xanthelesma and proptosis.9 The variety of orbital man- cardiac, or renal disease.14 Pulmonary function tests in ifestations includes infiltration of the fat and optic sheath patients with Erdheim-Chester typically indicate moderate to large retrobulbar intraconal masses.7 These symptoms restrictive ventilatory defects with normal or reduced are usually verified with CT contrast of the head and face Erdheim-Chester disease is typically confirmed by radiol- ogy and histology findings. Radiography is used to identifypleural and bone changes that are common to the white Erdheim-Chester Disease Medical Treatment Options cellYmediated group of diseases that are categorized as his- tiocytoses.15 This group of diseases is pathologically iden-tified by an infiltrate of lipid-laden foamy macrophages, which are the histiocytes or chronic inflammatory cells that lead to fibrosis.16 Histology can further character- Combination of prednisolone and clodronate ize the foamy macrophages, distinguishing them from Langerhans cells by using special staining techniques;positive results on staining for negative S-100 protein and Combination of cladribine and prednisolone CD1a indicate Erdheim-Chester disease.15 Although Copyright @ 2011 Lippincott Wil iams & Wilkins. Unauthorized reproduction of this article is prohibited.
there is neither sensitivity nor specificity reported for S-100 proteins are used for tumor markers. In the case of either radiological or histological tests, experts com- Erdheim-Chester disease, the presence of Langerhans cells monly cite specific findings from these diagnostic tests in allows for differential between Langerhans cells and nonYLangerhans cell histiocytosis.19 CD68 is a trans-membrane glycoprotein of unknown function highly ex- pressed by human monocytes and tissue macrophages.
Radiographic findings help differentiate between Erdheim- CD1a is a group of glycoproteins, which are often ex- Chester and Langerhans cell histiocytosis. In the case of pressed as immature thymocytes, Langerhans cells, and diagnosing lung involvement, plain chest films often show dendritic cells. When S-100 and CD1a are negative, the pleural thickening and pleural effusions in Erdheim-Chester histology confirms Erdheim-Chester disease mostly disease. Computed tomography of the chest predominately related to the negative test for Langerhans cells.20 shows smooth interlobular septal and visceral pleuralthickening predominately in the upper lobes of the lungs.
In regard to diagnosis of other body system involvement, CT The differential diagnosis for Erdheim-Chester disease is findings are associated with histopathologic changes and broad due to multisystem involvement and rarity of the cellular aggregation and fibrosis.3 The criterion-standard disease. The age of the patient should be considered as diagnostic test used to confirm Erdheim-Chester disease is Erdheim-Chester disease is rarely diagnosed in children.3 The long-bone x-ray. These x-rays show diffuse sclerosis primary differential diagnosis should include Langerhans throughout the metaphyses and diaphyses of the long cell histiocytosis. Both Erdheim-Chester disease and bones of the upper and lower extremities. Trabecular Langerhans cell histocytosis manifest with cellular infil- coarsening and cortical thickening produce osteosclerosis, tration of the bone, diabetes insipidus, and interstitial and this finding is diagnostic of Erdheim-Chester disease.3 lung disease3 (Table 2). To differentiate between the two,bone and pulmonary radiographs are used. In Erdheim-Chester disease, long-bone x-rays will show long-bone In regard to diagnosis of other body
osteosclerosis, whereas with Langerhans cell histiocytosis system involvement, CT findings are
skeletal osteolytic lesions are observed. In regard to thepulmonary radiology, Erdheim-Chester will often reveal associated with histopathologic
infiltrates to the perilymphangitic and subpleural lung changes and cellular aggregation and
tissue. In Langerhans cell histocytosis, tissues surrounding fibrosis.
According to the case study provided, the patient’s differential diagnosis included CHF exacerbation, intersti-tial pulmonary fibrosis, and adult respiratory disease syn- drome (ARDS). These diagnoses were explored related to A tissue biopsy can also diagnose Erdheim-Chester disease her course of progression. She initially presented with in- in addition to a positive long-bone x-ray. Bone or retro- creasing shortness of breath and a 30-lb unexplained weight orbital tissue has been the most beneficial area on which to gain. She had invasive hemodynamic monitoring as well as perform a biopsy in the confirmation of Erdheim-Chester.
an echocardiogram to rule out CHF. Her pulmonary status The lesions that are biopsied generally consist of lipid- was very difficult to differentiate. From an ARDS stand- storing CD68-positive and S-100Y and CD1a-negative, as point, she had bilateral pulmonary effusions, PaO2/FiO2 well as nonYLangerhans cell histiocytes, either localized to ratio less than 200, and noncardiogenic pulmonary ede- the bone or involving multiple organ systems in the body.17 ma. Her oxygen requirements and positive end-expiratory In the case of lung involvement, obtaining a transbron- pressure improved once a stress dose steroid was initiated chial or open lung biopsy of the affected tissue will often for possible idiopathic pulmonary fibrosis. Idiopathic pul- confirm nonYLangerhans cell histiocytic infiltrates.3 The monary fibrosis is evaluated by daily chest x-rays. Because absence of Birbeck granule or the presence of immunos- treatment was not improving her symptoms from a pul- taining negative for S-100 protein is often found. S-100 monary standpoint, it was decided to do an open lung cells are derived from the neural cells, which include biopsy to rule out any other possible treatable diseases.
Langerhans cells, schwann cells, glial cells, and melano-cytes. The function of S-100 cells, which are proteins that regulate protein phosphorylation, transcription factors, Pertaining to the case study provided, it was difficult to calcium homeostasis, enzyme activities, and cell growth arrive at the diagnosis. The differential diagnoses included are linked in the inflammatory response.18 Several of the were CHF exacerbation, idiopathic pulmonary fibrosis, Copyright @ 2011 Lippincott Wil iams & Wilkins. Unauthorized reproduction of this article is prohibited.
and ARDS. After several days of requiring mechanical combination therapy including prednisolone and a chemo- ventilation and failure to wean off of mechanical ventila- therapeutic agent is successful in a few cases.
tion, a decision was made to take an open lung biopsy of amass noticed on the chest x-ray. After investigation of themass and identification of a negative S-100 protein wasidentified from the lung tissue biopsy, long-bone x-rays There are other studies investigating
confirmed Erdheim-Chester disease with noticeable dif- different treatment options.
fuse sclerosis throughout the metaphyses and diaphyses ofthe lower extremities. At this time, hematology and rheu-matology consultations were made for treatment andmanagement of Erdheim-Chester disease.
A study done by Bourke and colleagues15 reviewed a patient who received combination therapy that included cyclophosphamide and prednisolone. The patient origi- Treatment is on an individual basis (Table 3). There have nally received prednisolone alone, and when taken off of been no randomized controlled trials in the literature. Un- the medication, the patient’s condition declined rapidly.
fortunately, 60% of patients with a diagnosis of Erdheim- Once the patient was restarted on prednisolone and the Chester expire within 32 months of presentation.14 A cyclophosphamide was initiated simultaneously, the pa- standardized treatment regimen of Erdheim-Chester has tient showed improvements in symptoms, function, and not been implicated because the history of this rare dis- radiological findings and slowed rate of progression.15,22 ease is not well known. Treatment options have included Another potential treatment option studied is cladribine, a surgical resection of lipid mass, corticosteroid therapy, purine analog that is toxic to monocytes.22 A study done cytotoxic agents, stem cell transplantation, radiation, and in 1999 investigated the use of this drug on a patient with interferon.3,14 Corticosteroids are the first line of treat- orbital and pericardial involvement.22 This patient also ment used to control symptoms but are not indicated for received steroids over a period, and when the dose was long-term management because of their potential for se- tapered off, his symptoms worsened. After restarting the rious adverse effects of long-term use.5 There are studies prednisolone and initiating cladribine, the patient showed investigating different treatment options. One study in- increasing monocyte production and a significant improve- vestigated the use of interferon " on 3 patients with di- ment in symptoms. The long-term effects of the treatment abetes insipidus and vision impairments. The interferon are not well known, but this patient had a good quality of " had a long-lasting response that improved outcomes for life for 2 years after treatment was discontinued.22 3 to 4.5 years.1 The use of bisphosphonate has beenstudied with bone involvement for Erdheim-Chester dis- ease. This line of drug choice is suggested to work well be- When assessing a patient who has decreasing pulmonary cause of its antimacrophage activity.21 Authors of a study function that does not improve with treatment, critical- noted that the use of combination therapy of oral pred- care nurses should be aware of the possibility of this rare nisone and intravenous clodronate over 5 years decreased disease. There are several different treatment options avail- the turnover of bone markers in their patients.21 Because able for Erdheim-Chester disease, all of which do not have of the rarity of pulmonary involvement, treatment is not enough evidence to support definitive treatment. More re- well established. It is suggested in literature that the use of search is needed in this area. It is important to treat thepatient based on presenting symptoms and system involve-ment. Steroids are the suggested initial treatment for symp-tom management and slow the rate of progression but are not recommended for long-term use. Combination ther- apy of steroids and a chemotherapeutic agent seems to be the most successful treatment pertaining to longer lifeexpectancy and symptom management at this time.
1. Braiteh F, Boxrud C, Esmaeli B, Kurzrock R. Successful treat- ment of Erdheim-Chester disease, a nonYLangerhans-cell histio-cytosis with interferon-alpha. Blood. 2005;106(9):2992-2994.
2. Devouassoux G, Lantuegoul S, Chatelain P, Brambilla E, Brambilla C. Erdheim-Chester disease: a primary macrophage cell disorder. Am J Respir Crit Care Med. 1998;157:650-653.
3. Shamburek D, Brewer B, Guchuico B. Erdheim-Chester Copyright @ 2011 Lippincott Wil iams & Wilkins. Unauthorized reproduction of this article is prohibited.
disease: a rare multisystem histiocytic disorder associated with 5. Devouassoux G, Lantuejoul S, Chatelain P, Brambilla E, interstitial lung disease. Am J Med Sci. 2001;321(1):66-75.
Brambilla C. Erdheim-Chester disease a primary macrophage 4. Chung J, Park M, Shin D, et al. Pulmonary involvement in cell disorder. Am J Respir Crit Care Med. 1998;157:650-653.
Erdheim-Chester disease. Respirology. 2005;10(3):389-392.
6. Donato R. Intracellular and extracellular roles of S100 pro- 5. Veyssier-Belot C, Cacoub P, Caparros-Lefebvre K, et al.
teins. Microsc Res Tech. 2003;60(6):540-551.
Erdheim-Chester disease: clinical and radiographic character- 7. Eyigor S, Kirazh Y, Memis A, Basdemir G. Erdheim-Chester istics of 59 cases. 1996;75(3):157-169.
disease: the effect of bisphosponate treatmentVa case report.
6. Stanway A. DermNet NZ. NonYLangerhans cell histiocytosis.
Arch Phys Med Rehabil. 2005;86:1053-1057.
http://dermnetnz.org/dermal-infiltrative/non-langerhans.html.
8. Fortman B. Erdheim-Chester disease of the retroperitoneum: a rare casuse of ureteral obstruction. AJR. 2001;176:1330-1331.
7. Passalaqua S, Janicek M. Erdheim-Chester disease. Joint Pro- 9. Haroche J, Amoura Z, Touraine P, et al. Bilateral adrenal in- gram in Nuclear Medicine. http://www.med.harvard.edu/JPNM/ filtration in Erdheim-Chester disease. Report of seven cases TF00-01/Oct 3/WriteUp.html. Accessed March 4, 2011.
and literature review. J Clin Endocrinol Metab. 2007;92(6): 8. Sistermann R, Katthagan D. Erdheim-Chester disease: a rare case of knee and leg pain. Arch Orthop Trauma Surg. 2000:112-113.
10. Hunger R, Sieling P, Ochoa M, et al. Langerhans cells utilize 9. Lau W, Chan E, Chan C. Orbital involvement in Erdheim- CD1a and langerin to efficiently present nonpeptide antigens Chester disease. Hong Kong Med J. 2007;13:238-240.
to T cells. J Clin Investig. 2004;113(5):658-660.
10. Fortman B. Erdheim-Chester disease of the retroperitoneum: 11. Kong P, Pinheiro L, Kaw G, Sittampalam K, Teo C. Erdheim- a rare cause of ureteral obstruction. Am J Roentgenol. 2001; Chester disease: a rare cause of interstitial lung disease.
11. Haroche J, Amoura Z. Touraine P, et al. Bilateral adrenal in- 12. Lau W, Chan E, Chan C. Orbital involvement in Erdheim- filtration in Erdheim-Chester disease: report of seven cases and Chester disease. Hong kong Med J. 2007;13:238-240.
literature review. J Clin Endocrinol Metab. 2007;92(6):1-12.
13. Myra C, Sloper L, Tighe P. Treatment of Erdheim-Chester dis- 12. Protopapadakis C, Antoniou K, Nicholson A, et al. Erdheim- ease with cladribine: a rational approach. Br J Opthamol. 2004; Chester disease: pulmonary presentation in a case with ad- vanced systemic involvement. Respiration. 2009;77:337-340.
14. Nonaka D, Chiriboga L, Rubin B. Differential expression of 13. REDORBIT.COM. Pulmonary and ophthalmic involvement S100 protein subtype in malignant melinoma, and benign and with Erdheim-Chester disease: a case report and review of the malignant periphreal nerve sheath tumors. J Cutan Pathol.
literature. 2004. www.redorbit.com/modules/news/tools.php.
15. Passalaqua S, Janicek M. Erdheim-Chester disease. Joint Pro- 14. Kong P, Pinheiro L, Kaw G, Sittampalam K, Teo C. Erdheim- gram in Nuclear Medicine. 2000. http://www.med.harvard.
Chester disease: a rare case of interstitial lung disease.
edu/JPNM/TF00_01/Oct3/WriteUp.html. Accessed March 4, 15. Bourke S, Nicholson A, Gibson G. Erdheim-Chester disease: 16. Protopapadakis C, Antoniou K, Nicholson A, et al. Erdheim- pulmonary infiltration responding to cyclophosphamide and Chester disease: pulmonary presentation in a case with ad- prednisolone. Thorax. 2003;58:1004-1005.
vanced systemic involvement. Respiration. 2009;77:337-340.
16. Wittenburg K, Swensen S, Myers J. Pulmonary involvement 17. REDORBIT.COM. Pulmonary and opthalmic involvement with with Erdheim-Chester disease: radiographic and CT findings.
Erdheim-Chester disease: a case report and review of the literature. 2004. www.redorbit.com/modules/news/tools.php.
17. Al-quaran S, Rieth S, Bradley J, Rimsza L. Erdheim-Chester disease: case report, PCR based analysis of clonality and re- 18. Shamburek D, Brewer B, Gochuico B. Erdheim-Chester view of literature. Mod Pathol. 2002;11(6):666-672.
disease: a rare multisystem histiocytic disorder associated with 18. Donato R. Intracellular and extracellular roles of S100 pro- interstitial lung disease. Am J Med Sci. 2001;321(1):66-75.
teins. Microsc Res Tech. 2003;60(6):540-551.
19. Sheu S, Wenzel R, Kersting C, Merten R, Otterbach F, Schmid K.
19. Nonaka D, Chiriboga L, Rubin B. Differential expression of Erdheim-Chester disease: case report with multisystemic mani- S100 protein subtype in malignant melanoma, and benign and festation including testes, thyroid, and lymph nodes, and a review malignant peripheral nerve sheath tumors. J Cutan Pathol.
of literature. J Clin Pathol. 2004;57:1225-1228.
20. Sistermann R, Katthagen D. Erdheim-Chester disease: a rare 20. Hunger R, Sieling P, Ochoa M, et al. Langerhans cells utilize cause of knee and leg pain. Arch Orthop Trauma Surg. 2000; CD1a and langerin to efficiency present nonpeptide antigens to T cells. J Clin Investig. 2004;113:658-660.
21. Stanway A. DermNet NZ. NonYLangerhans cell histiocytosis.
21. Eyigor S, Kirazh Y, Memis A, Basdemir G. Erdheim-Chester 2009. http://dermnetnz.org/dermal-infiltrative/non-langerhans.
disease: the effect of bisphosphonate treatmentVa case report.
Arch Phys Med Rehabil. 2005;86:1053-1057.
22. Veyssier-Belot C, Cacoub P, Caparros-Lefebvre D, et al.
22. Myra C, Sloper L, Tiche T. Treatment of Erdheim-Chester Erdheim-Chester disease: clinical and radiographic character- disease with cladribine: a rational approach. Br J Ophthalmol.
istics of 59 cases. Medicine. 1996;75(3):157-169.
23. Wittenberg K, Swensen S, Myers J. Pulmonary involvement with Erdheim-Chester disease: radiographic and CT findings.
1. Al-quran S, Reith S, Bradley J, Rimsza L. Erdheim-Chester disease: case report, PCR based analysis of clonality, andreview of literature. Mod Pathol. 2002;15:666-672.
2. Bourke S, Nicholson A, Gibson G. Erdheim-Chester disease: pulmonary infiltration responding to cyclophosphamide and Kevin Andrysek, MSN, ACNP-C, CCRN, is a Flight Acute Care Nurse prednisolone. Thorax. 2003;58:1004-1005.
Practitioner at Cleveland Clinic Critical Care Transport, Cleveland 3. Braiteh F, Boxrud C, Esmaeli B, Kurzrock R. Successful treat- Clinic Foundation. He finished his BSN at Malone University and MSN ment of Erdheim-Chester disease, a non-Langerhans-cell histio- at Case Western Reserve University. He also attended the National cytosis, with interferon-alpha. Blood. 2005;106(9):2992-2994.
4. Chung J, Park M, Shin D, et al. Pulmonary involvement in Erdheim-Chester disease. Respirology. 2005;10(3):389-392.
Address correspondence and reprint requests to: (Kaa66@case.edu).
Copyright @ 2011 Lippincott Wil iams & Wilkins. Unauthorized reproduction of this article is prohibited.

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