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Carrington Syndrome

Chronic Eosinophilic Pneumonia (cep)

Carrington syndrome is a rare idiopathic disorder characterized by eosinophilic infiltration into the lungs, causing chronic, recurrent respiratory symptoms. It has a female preponderance and is mostly an adult illness.


Presentation

Carrington syndrome (CS) is a rare interstitial lung ailment that is progressive in nature [1]. The disease process is caused by an accumulation of eosinophils in the lungs, thus it is also called chronic eosinophilic pneumonia. The etiology of CS is unclear, although it may be linked to allergic reactions. It predominantly affects women, usually manifesting in middle-aged patients [2]. Of the known cases of CS, few of them involve children [3]. Furthermore, only about 10% of cases have a history of tobacco use [4]. CS is considered chronic, however clinically, the disease is characterized by recurrent acute and sub-acute exacerbations. It is reported that up to 50% of affected individuals also suffer from asthma [5].

Symptoms of CS during an episode are often subclinical, consisting of respiratory complaints such as coughing, wheezing, dyspnea and crepitations on auscultation. As a result, diagnosis may only be made weeks after onset. The dyspnea experienced may be minimal and in some cases severe [6]. However, patients are rarely ill enough to require assisted ventilation [7]. The overall clinical impression may be similar to that of community-acquired pneumonia. Non-pulmonary signs, when present, are non-specific and frequently include fever, weight loss, night sweats and generalized body weakness. Conversely, the presence of systemic pathology is not typical of CS and may prompt the consideration of an alternative diagnosis, namely, idiopathic hypereosinophilic syndrome (IHS) [8].

Weight Loss
  • Non-pulmonary signs, when present, are non-specific and frequently include fever, weight loss, night sweats and generalized body weakness.[symptoma.com]
  • Symptoms usually associated fever, weight loss, fatigue and dyspnoea. Extra-respiratory manifestations are rare but may involve many organs.[ncbi.nlm.nih.gov]
  • The symptoms are weakness, malaise, progressive weight loss, purulent rhinitis, sinusitis, polyarthralgia, ulcerations of the nasal septum, signs of severe progressive renal disease, and fever. The disease commonly occurs in mid-adult life.[whonamedit.com]
  • Symptoms accumulate over several months and include fevers, cough, breathlessness, wheezing, and weight loss.[medigoo.com]
Anemia
  • Laboratory studies include: Full blood count: This has diagnostic value as it may reveal thrombocytosis, high eosinophil count (over 1000/mm3), and iron deficiency anemia, which are associated with CS.[symptoma.com]
  • Laboratory tests typical of chronic eosinophilic pneumonia include increased levels of eosinophils in the blood, a high erythrocyte sedimentation rate, iron deficiency anemia, and increased platelets.[en.wikipedia.org]
  • Adverse Reactions:Local reaction to anaphylaxis Mechanism/Use: Inhibit DNA synthesis/renal rejection, rheumatoid arthritis Adverse Reactions:N/V, thrombocytopenia, anemia, alopecia Cytotoxic; Cyclophosphamide Mechanism/Use: Alkylating agent/rheumatic[quizlet.com]
Malaise
  • […] chronic eosinophilic pneumonia (ICEP) is a very rare, severe, interstitial lung disease of insidious onset with subacute or chronic non-specific respiratory manifestations (dyspnea, cough, wheezing) often associated with systemic manifestations (fatigue, malaise[rarediseases.info.nih.gov]
  • The symptoms are weakness, malaise, progressive weight loss, purulent rhinitis, sinusitis, polyarthralgia, ulcerations of the nasal septum, signs of severe progressive renal disease, and fever. The disease commonly occurs in mid-adult life.[whonamedit.com]
  • Before these severe findings develop, people often have a flu-like prodrome , with a cough, runny nose, fever, decreased appetite and malaise .[en.wikipedia.org]
Crying
  • I went to dress Carrington for her bath and when I removed her clothing what I saw caused me to cry out in extreme disgust.[adoptionnutrition.org]
Chronic Fatigue Syndrome
  • According to Tech Times, chronic fatigue syndrome affects between one and four million people, but estimating a more exact number can’t be generated because the condition can’t be easily diagnosed. 1 Chronic fatigue syndrome According to the Centers for[carrington.edu]
Pleural Effusion
  • Pleural effusion is not usually noticed, and pulmonary lesions may be migratory. Computerized tomography (CT) scan: At high resolution, areas of consolidation, ground glass lesions, and other opacities are typically seen.[symptoma.com]
  • In pleural effusion may be observed. Treatment and prognosis The long-term prognosis is considered excellent but the majority often will require long-term low-dose oral corticosteroid therapy in order to prevent relapse 7-8.[radiopaedia.org]
  • Pulmonary involvement is observed in 40% of cases and is characterized by interstitial infiltrates on chest imaging; pleural effusion is seen in 50% of affected patients [ 32 ].[link.springer.com]
  • effusion: a case report. ( 27520469 ) Sriratanaviriyakul N....Albertson T.E. 2016 18 A CASE OF EOSINOPHILIC BRONCHIOLITIS COMPLICATED WITH BRONCHIAL ASTHMA AND CHRONIC EOSINOPHILIC PNEUMONIA. ( 27086960 ) Sakai H....Ishizuka T. 2016 19 The Long-term[malacards.org]
  • Radiological abnormalities are solitary or multiple nodules or masses ( figure 4a ), with areas of airspace consolidation or ground-glass attenuation, mediastinal lymphadenopathy and pleural effusions.[err.ersjournals.com]
Sore Throat
  • Symptoms of the condition include unrefreshing sleep, muscle pain, sore throat, head ache, and even potential lapses in short term memory. In the past, the process of diagnosing CFS required ruling out any other possible medical condition.[carrington.edu]
Rales
  • On chest auscultation few end inspiratory rales were audible in eight patients (14%). Table 1 shows the pulmonary function data.[ard.bmj.com]
Macroglossia
  • "— Presentation transcript: 1 p57: Beckwith-Wiedemann Syndrome Presented By: Jameeka Carrington 2 Symptoms of BWS  Large body size (macrosomia)  Large tongue (macroglossia)  Large organs (visceromegaly)  Abdominal wall defects (i.e. umbilical hernia[slideplayer.com]
Tachycardia
  • "Postural orthostatic tachycardia syndrome," said Primary Children's Hospital Pediatric Cardiologist Dr. Susan Etheridge. It's a mouthful and it meant big changes for 13-year-old Emma. "Dance was my life. I did dance competitions.[good4utah.com]
  • Nonetheless, signs and management of a major bleed are as follows: Tachycardia may be the first sign of bleeding – take it seriously Classic signs of shock and back pain may happen much later If shock develops call your blood bank and X-match 2 (or more[renalmed.co.uk]
  • Limitations: While quite safe, toxicity will frequently include nervousness, tremors, and occasionally seizures, tachycardia, dysrrhythmias. Does increase SVR probably through catecholamine release. Nausea and vomiting can occur.[quizlet.com]
Vascular Disease
  • In separate chapters, the major connective tissue diseases are reviewed, with detailed discussion of a variety of lung abnormalities, including pleural disease, parenchymal lung disease and pulmonary vascular disease.[books.google.com]
  • The relation of vascular disease to the hypertensive state. JAMA 1943; 121: 1256-1261 Diamantis A et al. Fine-needle aspiration (FNA) biopsy: historical aspects. Folia Histochem Cytobiol. 2009; 47(2): 191-7 Edebohls, GM.[renalmed.co.uk]
  • Acute complications of DM Diabetic ketoacidosis (DKA) Hyperglycemic, hyper osmolar non-ketotic coma (HHNC) Hypoglycemia Chronic complications of DM Nephropathy Retinopathy Neuropathy Peripheral vascular disease Monitoring of blood glucose Urine vs. blood[quizlet.com]
Forgetful
  • Towanda, PA – We hear people say Thank You so many times a day that it gets easy to forget how truly special a thank you is. For Flynn Energy & Propane president EJ Flynn, a simple Thank You has the power to change a life.[flynn-energy.com]
  • It just seemed to come out of the air: “I choose to NOT CARE AT ALL what Cindy and the kids think and go about my own life and forget about them.” “On the right track, but it still needs a little work.” Pat said.[patcarrington.com]
  • Don’t forget that we all emit light from our bodies but at a level 1,000 times below the threshold of the eye.[alternative-doctor.com]
Headache
  • Other symptoms include difficulty concentrating, confusion, and headache (CNS dysfunction).[quizlet.com]
  • Clin Evid (Online). 2010 Jul 19;2010. pii: 1301 International headache society Classification of BMS ihs-classification.org/en/02.../04_teil3/13.18.05_facialpain.html Grushka M. Clinical features of burning mouth syndrome.[orofacialpain.org.uk]
Average Intelligence
  • الصفحة 14 - This definition (of /earning disability) covers adults with autism ...but not those who may be of average or even above average intelligence, such as people with Asperger syndrome. ‏[books.google.com]

Workup

Diagnosis of CS is made on the basis of clinical, laboratory and radiological evidence. Clinical features may be ambiguous, thus the latter studies are useful in delineating the etiology.

Laboratory studies include:

  • Full blood count: This has diagnostic value as it may reveal thrombocytosis, high eosinophil count (over 1000/mm3), and iron deficiency anemia, which are associated with CS.
  • Inflammatory markers: Usually the erythrocyte sedimentation rate (ESR) and C-reactive protein are high (CRP).
  • Iron studies.
  • Arterial blood gas analysis may show reduced oxygen levels [9].
  • Blood culture: This rules out possible infectious causes.
  • Bronchoalveolar lavage (BAL): BAL may show over 40% eosinophils on a differential count, although only 20% is needed for alveolar inflammation to occur. Nevertheless, a value in excess of 1% is abnormal [2]. BAL is useful when there is no convincing evidence of CS from blood eosinophil counts and chest radiographs. Furthermore, certain histological findings on the analysis of lung samples, are indicative of CS, that is, lung exudates, bronchiolitis, granuloma formation, and organizing pneumonia [10].
  • Pulmonary function tests (PFT): Either obstructive or restrictive lung disease patterns may be demonstrated, the latter especially seen in those with pre-existing asthma. One in three patients has normal PFT results.

Imaging studies:

  • Chest X-rays in about 50% of patients show bilateral infiltrative lung disease that is more prominent in the upper lobe and around the outer edges of the lung fields, giving the appearance of a photographic negative of acute pulmonary edema [11]. Pleural effusion is not usually noticed, and pulmonary lesions may be migratory [4].
  • Computerized tomography (CT) scan: At high resolution, areas of consolidation, ground glass lesions, and other opacities are typically seen. These lesions are also found in sarcoidosis and various pneumonia [8].
Thrombocytosis
  • Laboratory studies include: Full blood count: This has diagnostic value as it may reveal thrombocytosis, high eosinophil count (over 1000/mm3), and iron deficiency anemia, which are associated with CS.[symptoma.com]
  • Markers increased serum IgE levels can be seen in two-thirds of patients the erythrocyte sedimentation rate (ESR) is usually elevated peripheral blood thrombocytosis has also been reported the percentage of eosinophils in the BAL fluid is very high Plain[radiopaedia.org]
  • […] classical radiographic pattern of chronic eosinophilic pneumonia. ( 26155798 ) Cherian S.V....Thampy E. 2015 22 Efficacy of short-term prednisolone treatment in patients with chronic eosinophilic pneumonia. ( 25614171 ) Oyama Y....Suda T. 2015 23 Marked Thrombocytosis[malacards.org]
Chlamydia
  • Current knowledge on Chlamydia pneumoniae, strain TWAR, an important cause of pneumonia and other acute respiratory diseases. Eur J Clin Microbiol Infect Dis 1989;8: 191-202. ‏ الصفحة 44 - Nieto FJ, Adam E, Sorlie P, et al.[books.google.com]
Visceromegaly
  • "— Presentation transcript: 1 p57: Beckwith-Wiedemann Syndrome Presented By: Jameeka Carrington 2 Symptoms of BWS  Large body size (macrosomia)  Large tongue (macroglossia)  Large organs (visceromegaly)  Abdominal wall defects (i.e. umbilical hernia[slideplayer.com]
Pleural Effusion
  • Pleural effusion is not usually noticed, and pulmonary lesions may be migratory. Computerized tomography (CT) scan: At high resolution, areas of consolidation, ground glass lesions, and other opacities are typically seen.[symptoma.com]
  • In pleural effusion may be observed. Treatment and prognosis The long-term prognosis is considered excellent but the majority often will require long-term low-dose oral corticosteroid therapy in order to prevent relapse 7-8.[radiopaedia.org]
  • Pulmonary involvement is observed in 40% of cases and is characterized by interstitial infiltrates on chest imaging; pleural effusion is seen in 50% of affected patients [ 32 ].[link.springer.com]
  • effusion: a case report. ( 27520469 ) Sriratanaviriyakul N....Albertson T.E. 2016 18 A CASE OF EOSINOPHILIC BRONCHIOLITIS COMPLICATED WITH BRONCHIAL ASTHMA AND CHRONIC EOSINOPHILIC PNEUMONIA. ( 27086960 ) Sakai H....Ishizuka T. 2016 19 The Long-term[malacards.org]
  • Radiological abnormalities are solitary or multiple nodules or masses ( figure 4a ), with areas of airspace consolidation or ground-glass attenuation, mediastinal lymphadenopathy and pleural effusions.[err.ersjournals.com]

Treatment

  • Treatment - Carrington syndrome Resources - Carrington syndrome[checkorphan.org]
  • […] starts and/or until lesions are crusted or healed Antibiotic treatment by mouth may speed healing.[carrington.nottingham.sch.uk]
  • Reflexology can be successfully used to support the treatment of many illnesses and conditions.[carringtonpharmacy.co.uk]
  • Food and Drug Administration has chosen Wave Life Sciences’ planned phase 2/3 study of suvodirsen, its lead experimental therapy for the treatment of Duchenne muscular dystrophy, to break new ground.[globalgenes.org]
  • Treatment Options: Carrington Disease may be mild, and people with the disease may get better without treatment. For acute cases, a corticosteroid such as prednisone is usually needed.[medigoo.com]

Prognosis

  • Prognosis - Carrington syndrome Not supplied. Treatment - Carrington syndrome Resources - Carrington syndrome[checkorphan.org]
  • The exocrine manifestations, diagnosis, treatment, and prognosis of both sicca and extraglandular manifestations are discussed separately.[uptodate.com]
  • Treatment and prognosis The long-term prognosis is considered excellent but the majority often will require long-term low-dose oral corticosteroid therapy in order to prevent relapse 7-8.[radiopaedia.org]
  • Prognosis, sequelae, diagnosis, differential diagnosis, prevention, and treatment". Journal of the American Academy of Dermatology . 69 (2): 187.e1–16, quiz 203–4. doi : 10.1016/j.jaad.2013.05.002 .[en.wikipedia.org]

Etiology

  • Clinical features may be ambiguous, thus the latter studies are useful in delineating the etiology.[symptoma.com]
  • Although the etiology for these symptoms is unclear, it may be due to motor unit dysfunction manifested by deterioration of the peripheral axons and neuromuscular junction, probably as result of overwork.[dl.begellhouse.com]
  • Definition (MSH) A common interstitial lung disease of unknown etiology, usually occurring between 50-70 years of age.[fpnotebook.com]
  • This condition is similar but distinct from MRKH syndrome (see Etiology section) and may therefore lead to confusion.[ncbi.nlm.nih.gov]

Epidemiology

  • This article reviews the epidemiology, pathophysiology, characteristics, assessment, and rehabilitation care of the patient with PPS.[dl.begellhouse.com]
  • Long-term use of prednisone has many side effects, including increased infections, osteoporosis, stomach ulcers, Cushing's syndrome, and changes in appearance. [6] Epidemiology [ edit ] Eosinophilic pneumonia is a rare disease.[en.wikipedia.org]
  • Epidemiology Epidemiological data available on BMS is very limited.[orofacialpain.org.uk]
  • PubMed Google Scholar Martinez-Frias ML, Bermejo E, Rodriguez-Pinilla E, Prieto L, Frias JL: Epidemiological analysis of outcomes of pregnancy in gestational diabetic mothers.[ojrd.biomedcentral.com]
  • Epidemiological analysis of outcomes of pregnancy in gestational diabetic mothers.[ncbi.nlm.nih.gov]
Sex distribution
Age distribution

Pathophysiology

  • This article reviews the epidemiology, pathophysiology, characteristics, assessment, and rehabilitation care of the patient with PPS.[dl.begellhouse.com]
  • She co-authored Post-Polio Syndrome: Pathophysiology and Clinical Management with Lauro S.[post-polio.org]
  • Pathophysiology of primary burning mouth syndrome. Clin Neurophysiol. 2011 Oct 24 Albuquerque RJ, de Leeuw R, Carlson CR, Okeson JP, Miller CS, Andersen AH.[orofacialpain.org.uk]
  • Pathophysiology [ edit ] Eosinophilic pneumonia can develop in several different ways depending on the underlying cause of the disease. Eosinophils play a central role in defending the body against infection by parasites.[en.wikipedia.org]
  • Eugenidio Hospital, Athens, Greece, b Department of Internal Medicine, Medical School, University of Ioannina, Ioannina, Greece, c Department of Critical Care Medicine, Medical School, National and Kapodestrian University, Athens, Greece, d Department of Pathophysiology[ard.bmj.com]

Prevention

  • الصفحة 18 - Prevention of coronary heart disease in clinical practice: recommendations of the Second Joint Task Force of European and other Societies on Coronary Prevention, Atherosclerosis 140:199-270, 1998. ‏[books.google.com]
  • […] chronic fatigue syndrome affects between one and four million people, but estimating a more exact number can’t be generated because the condition can’t be easily diagnosed. 1 Chronic fatigue syndrome According to the Centers for Disease Control and Prevention[carrington.edu]
  • Prevention - Carrington syndrome Not supplied. Diagnosis - Carrington syndrome Not supplied. Prognosis - Carrington syndrome Not supplied. Treatment - Carrington syndrome Resources - Carrington syndrome[checkorphan.org]
  • This not only can help prevent the development of periodontal disease, but can make the entire body healthier.[carringtondentalcenter.ca]

References

Article

  1. Thomeer MJ, Costabel U, Rizzato G, Poletti V, Demedts M. Comparison of registries of interstitial lung diseases in three European countries. Eur Respir J Suppl. 2001;32:114s–118s.
  2. Sano S, Yamagami K, Yoshioka K. Chronic eosinophilic pneumonia: a case report and review of the literature. Cases J. 2009;2:7735.
  3. Wubbel C, Fulmer D, Sherman J. Chronic eosinophilic pneumonia: a case report and national survey. Chest. 2003;123(5):1763–1766.
  4. Naughton M, Fahy J, Fitzgerald MX. Chronic eosinophilic pneumonia. A long-term follow-up of 12 patients. Chest. 1993;103(1):162–165.
  5. Marchand E, Etienne-Mastroianni B, Chanez P, et al. Idiopathic chronic eosinophilic pneumonia and asthma: how do they influence each other? Eur Respir J. 2003;22(1):8–13.
  6. Marchand E, Reynaud-Gaubert M, Lauque D, Durieu J, Tonnel AB, Cordier JF. Idiopathic chronic eosinophilic pneumonia. A clinical and follow-up study of 62 cases. The Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM"O"P). Medicine (Baltimore). 1998;77(5):299–312.
  7. Libby DM, Murphy TF, Edwards A, Gray G, King TK. Chronic eosinophilic pneumonia: an unusual cause of acute respiratory failure. Am Rev Respir Dis. 1980;122(2):497–500.
  8. Marchand E, Cordier J-F. Idiopathic chronic eosinophilic pneumonia. Orphanet J Rare Dis. 2006;1:11.
  9. Durieu J, Wallaert B, Tonnel AB. La pneumonie chronique à éosinophiles ou maladie de Carrington. Rev Mal Respir. 1993;10(6):499–507.
  10. Jederlinic PJ, Sicilian L, Gaensler EA. Chronic eosinophilic pneumonia. A report of 19 cases and a review of the literature. Medicine (Baltimore). 1988;67(3):154–162.
  11. Olopade CO, Crotty TB, Douglas WW, Colby TV, Sur S. Chronic eosinophilic pneumonia and idiopathic bronchiolitis obliterans organizing pneumonia: comparison of eosinophil number and degranulation by immunofluorescence staining for eosinophil-derived major basic protein. Mayo Clin Proc. 1995;70(2):137–142.

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Last updated: 2019-06-28 11:17