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].
Entire Body System
- Swelling
Carpal tunnel syndrome is more common in pregnancy as the increased volume of blood and other body fluids can create swelling in the hands. When this swelling occurs in the wrist, it can compress the median nerve. [lifecyclephysio.com.au]
The most common side effects include dizziness, rapid heartbeat, throat swelling, and weakness or difficulty breathing. If there are any signs of side effects, be sure to visit the doctor or emergency room if these symptoms do not quickly subside. [carrington.edu]
The joint pains are only rarely accompanied by swelling and/or inflammation, but do demonstrate tenderness and abnormal range of motion. [post-polio.org]
Important skeletal features include brachydactyly and brachytarsia, deviation of the proximal and (less frequently) of the distal phalanges, painless swelling of the proximal interphalangeal joints, and hip changes. [jamanetwork.com]
In some cases, affected individuals may develop pain and limitation of movements in the hips and/or hands; inflammation and swelling (arthritis) in the fingers, elbows, and/or spine may also develop. [rarediseases.org]
- Pathologist
Wegener's granulomatosis, or arteritis-pulmonary-nephropathy syndrome, under Friedrich Wegener, German pathologist, 1907-1990. Bibliography C. B. Carrington, A. A. Liebow: Limited forms of angiitis and granulomatosis of Wegener’s type. [whonamedit.com]
After an initial review by 10 pathologists who were experienced in lung pathology (see Acknowledgment), two of the pathologists (M.K. and A.G.N.) further reviewed all pathologic specimens from 33 cases (31 surgical lung biopsies [SLB] and 2 autopsies) [atsjournals.org]
Pediatricians, dental specialists, speech pathologists, orthopedic surgeons, physicians who evaluate and treat skin problems (dermatologists), and other health care professionals may need to systematically and comprehensively plan an affected child's [rarediseases.org]
- Hodgkin Lymphoma
Pulmonary lymphoma Sjögren's syndrome is associated with a 16- to 44-fold increased risk of non-Hodgkin lymphoma [121]. Marginal zone B-cell lymphoma and mucosa-associated lymphoid tissue are the most common subtypes. [err.ersjournals.com]
Four patients showed non-Hodgkin lymphomas of mucosa-associated lymphoid tissue (MALT) origin. Two patients showed deposition of amyloid in the lung parenchyma as the major pattern. [atsjournals.org]
Because its clinical symptoms are generally mild or absent, its prognosis is favorable; only a small percentage of lesions progress to diffuse large B-cell non-Hodgkin lymphoma (20,32,33). [pubs.rsna.org]
A 32-year-old male patient is to undergo radiation therapy to the pelvic area for Hodgkin's lymphoma. He expresses concern to the nurse about the effect of chemotherapy on his sexual function. [quizlet.com]
- Malnutrition
Robert Jennings made this last statement. 18 minutes later he was dead. 31 Jan, 2019 2:15pm 4 minutes to read US senator thinks the world isn't taking the threat serious enough. 31 Jan, 2019 9:09am 2 minutes to read An autopsy shows the baby boy died of malnutrition [nzherald.co.nz]
Inflammation also is responsible for other mortality risk factors, such as anemia, malnutrition, vascular disease, and left ventricular hypertrophy. [jasn.asnjournals.org]
Carrington’s brain scan showed that she had agenesis of the corpus collosum and that her brain had shrunk due to malnutrition. Carrington was not able to take in any nutrition by mouth so an NG tube had to be placed. [adoptionnutrition.org]
[…] cancer is seen in these individuals, with cumulative lifetime risk of 50%. [37] Cronkhite-Canada syndrome has an extremely unfavorable prognosis, with a 5-year mortality rate of 55%, secondary to life-threatening GI bleeding, intussusception, infection, malnutrition [emedicine.medscape.com]
- Pallor
Full Text An 81-year-old male was hospitalized for fatigue, pallor and transfusion-dependent anemia. His physical exam was negative for lymphadenopathy or splenomegaly. [htct.com.br]
During January 2016, she developed pallor, jaundice, and lethargy. She was afebrile and had hepatosplenomegaly. Liver was palpable 10 cm below the subcostal margin, and the spleen was palpable 8 cm below thesubcostal margin. [oatext.com]
Respiratoric
- Pneumonia
In the case of eosinophilic pneumonia, the lungs are the target. Generally, eosinophilia pneumonia is not a reaction to an infection. There is a correlation between asthma and eosinophilic pneumonia. [medical-dictionary.thefreedictionary.com]
These lesions are also found in sarcoidosis and various pneumonia. [symptoma.com]
Idiopathic chronic eosinophilic pneumonia. [ojrd.biomedcentral.com]
Disease name and synonyms Idiopathic chronic eosinophilic pneumonia (ICEP); Chronic eosinophilic pneumonia (CEP); Carrington's disease. [link.springer.com]
Carrington's disease: idiopathic chronic eosinophilic pneumonia] [Article in French] C Piette et al. Rev Med Liege. 2014 Mar. [pubmed.ncbi.nlm.nih.gov]
- 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]
- Pulmonary Disorder
Pulmonary Manifestations The pulmonary manifestations of primary Sjögren syndrome encompass airway abnormalities, interstitial pneumonia, and lymphoproliferative disorders (1,2,9,11–13). [pubs.rsna.org]
Gastrointestinal
- Diarrhea
In patients undergoing cord-blood HSCT at our center, we have observed a syndrome of culture-negative, antibiotic-responsive diarrhea after engraftment that is not attributable to any of the aforementioned causes of diarrhea. [nejm.org]
Used to treat hypersecretory diarrhea, enteric fistulas. [quizlet.com]
Cardiovascular
- 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]
A physical examination may reveal low levels of oxygen in the blood (hypoxemia), a rapid heart rate (tachycardia), wheezes and a rattling sound in the lungs (rales). [rarediseases.org]
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]
Musculoskeletal
- Arthralgia
In addition to dryness, clinical presentation of Sjögren's syndrome generally includes asthenia and arthralgia. [err.ersjournals.com]
The frequency of symptoms experienced by patients during the follow-up period that were possibly related to the disease was as follows: sicca symptoms in 31 patients (94%), cough in 24 (73%), dyspnea in 21 (64%), arthralgia in eight (24%), fever in seven [atsjournals.org]
Skin
- Eruptions
Skin manifestations can include scarring, eruptive melanocytic nevi, vulvovaginal stenosis, and dyspareunia. [en.wikipedia.org]
[…] drug reactions but not with carbamazepine-induced lymphocyte proliferation in a Japanese population Hiroyuki NIIHARA,Takeyasu KAKAMU,Yasuyuki FUJITA,Sakae KANEKO,Eishin MORITA The Journal of Dermatology. 2012; 39(7): 594 [Pubmed] | [DOI] 78 Severe drug eruptions [ijdvl.com]
Neurologic
- Dizziness
SANDY, Utah (ABC 4 UTAH) - Two young Utah girls start to feel a bit dizzy, lightheaded and weak. But it's not the flu or the common cold. After a process of elimination, doctors discovered both have POTS syndrome. [good4utah.com]
The most common side effects include dizziness, rapid heartbeat, throat swelling, and weakness or difficulty breathing. If there are any signs of side effects, be sure to visit the doctor or emergency room if these symptoms do not quickly subside. [carrington.edu]
Montelukast: occasional reports of mild headache, fatigue, dizziness, GI upset, cough and elevation of transaminases (hepatic). Rare reports of severe behavior/mood changes, angioedema and heptatitis were found on post-marketing surveys. [quizlet.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].
Treatment
The symptoms occur gradually over a period of weeks or even months Symptoms Some of the symptoms of Carrington syndrome incude: * Cough * Fever * Breathing difficulty * Night sweats * Wheezing Treatment Treatments for Carrington syndrome (Idiopathic eosinophilic [checkorphan.org]
Symptoms and chest X-ray quickly improve under corticosteroid treatment. In the future, new research could lead to alternative treatments. We report the case of a woman with ICEP. [pubmed.ncbi.nlm.nih.gov]
[…] starts and/or until lesions are crusted or healed Antibiotic treatment by mouth may speed healing. [carrington.nottingham.sch.uk]
In an ideal world the treatment of surgical disorders of the thorax in children would be provided by a super specialist, but this is neither practical nor possible. [books.google.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]
Cordier Medicine Allergy 2005 The adjunction of immunosuppressants to corticosteroids is necessary in patients with CSS and poor prognosis factors, and Imatinib has recently proven effective in the treatment of the myeloproliferative variant of the HES [semanticscholar.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]
All rights reserved. eosinophilic pneumonia Clinical medicine A nonspecific term for disease processes in which the lung parenchyma is inflamed and diffusely infiltrated with eosinophils and histiocytes, ± PMNs, lymphocytes, plasma cells, mast cells Etiology [medical-dictionary.thefreedictionary.com]
Examine the role of genomics and pharmacogenomics in the etiology, treatment and eradication of preventable causes of drug-induced SJS/TEN. [genome.gov]
Definition (MSH) A common interstitial lung disease of unknown etiology, usually occurring between 50-70 years of age. [fpnotebook.com]
Epidemiology
Epidemiology of tinea capitis in Europe: current state and changing patterns. Mycoses. 2007;50:6-13. Havlickova B, Czaika VA, Friedrich M. Epidemiological trends in skin mycoses worldwide. Mycos. 2008;51:2-15. Ameen M. [ijord.com]
This article reviews the epidemiology, pathophysiology, characteristics, assessment, and rehabilitation care of the patient with PPS. [dl.begellhouse.com]
Jump to navigation Jump to search Pneumonia Main Page Eosinophilic pneumonia Microchapters Home Patient Information Overview Historical Perspective Classification Pathophysiology Causes Differentiating Eosinophilic pneumonia from other Diseases Epidemiology [wikidoc.org]
Clinical syndromes, epidemiology, genomics, diagnosis and treatment Video | Slides Neil Shear 9:30 a.m. Basic science of pathogenesis, functional genomics and mechanisms Video | Slides Wen-Hung Chung 10:00 a.m. [genome.gov]
Gloomier still, is the recent report from the Caribbean Epidemiology Centre (CAREC) that warns that the epidemic in the region is likely to escalate even further in the immediate future, since it is yet to reach its peak in most of our countries. [caricom.org]
Pathophysiology
This article reviews the epidemiology, pathophysiology, characteristics, assessment, and rehabilitation care of the patient with PPS. [dl.begellhouse.com]
Prevention remains the best treatment option given the high mortality rate and unclear pathophysiology. [hindawi.com]
Jump to navigation Jump to search Pneumonia Main Page Eosinophilic pneumonia Microchapters Home Patient Information Overview Historical Perspective Classification Pathophysiology Causes Differentiating Eosinophilic pneumonia from other Diseases Epidemiology [wikidoc.org]
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]
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]
Prevention - Carrington syndrome Not supplied. Diagnosis - Carrington syndrome Not supplied. Prognosis - Carrington syndrome Not supplied. Treatment - Carrington syndrome Resources - Carrington syndrome [checkorphan.org]
[…] 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]
References
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- Sano S, Yamagami K, Yoshioka K. Chronic eosinophilic pneumonia: a case report and review of the literature. Cases J. 2009;2:7735.
- Wubbel C, Fulmer D, Sherman J. Chronic eosinophilic pneumonia: a case report and national survey. Chest. 2003;123(5):1763–1766.
- Naughton M, Fahy J, Fitzgerald MX. Chronic eosinophilic pneumonia. A long-term follow-up of 12 patients. Chest. 1993;103(1):162–165.
- 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.
- 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.
- 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.
- Marchand E, Cordier J-F. Idiopathic chronic eosinophilic pneumonia. Orphanet J Rare Dis. 2006;1:11.
- Durieu J, Wallaert B, Tonnel AB. La pneumonie chronique à éosinophiles ou maladie de Carrington. Rev Mal Respir. 1993;10(6):499–507.
- 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.
- 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.