Congenital Ichthyosis and Neonatal Pulmonary Disease

PEDIATRICS ◽  
1974 ◽  
Vol 53 (4) ◽  
pp. 573-575
Author(s):  
M. Perlman ◽  
J. Bar-Ziv
Keyword(s):  
Respiratory Tract ◽  
Amniotic Fluid ◽  
Lung Disease ◽  
Pulmonary Disease ◽  
Fetal Distress ◽  
Pulmonary Aspiration ◽  
Congenital Ichthyosis ◽  
Pulmonary Manifestations ◽  
High Concentrations ◽  
Respiratory Movements

An atypical pulmonary aspiration syndrome occurred in two prematurely born siblings with congenital ichthyosis; pneumothorax and pneumomediastinum were seen in a third neonate with less severe ichthyosis. These pulmonary manifestations were probably complications of aspiration of amniotic fluid containing abnormally high concentrations of epithelial squames. The severity of the lung disease appeared to be correlated with the severity of the ichthyosis. Amniotic fluid enters the fetal respiratory tract either chronically, during physiological fetal respiratory movements or, acutely, by way of gasping initiated during fetal distress.

scholarly journals FRI0072 DISCONTINUATION OF DMARD USE IN RHEUMATOID ARTHRITIS PATIENTS WITH LUNG DISEASE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 612.1-613
Author(s):  
S. Pedro ◽  
T. Mikuls ◽  
J. Zhuo ◽  
K. Michaud
Keyword(s):  
Rheumatoid Arthritis ◽  
Lung Disease ◽  
Pulmonary Disease ◽  
Treatment Patterns ◽  
Dmard Treatment ◽  
Increased Risk ◽  
Pulmonary Manifestations ◽  
Clinical Models ◽  
Comorbidity Index ◽  
Time Varying Covariates

Background:Pulmonary manifestations such as interstitial lung disease (ILD) and chronic obstructive pulmonary disease (COPD) are frequent extra-articular features that carry a poor prognosis in Rheumatoid Arthritis (RA). Little data is available on how RA patients (pts) with pulmonary disease are managed in real-world settings.Objectives:To assess treatment patterns and DMARD discontinuation in RA patients with comorbid lung disease in comparison with other RA patients.Methods:The study included RA Patients enrolled in the Forward Databank with ≥1 year observation after 2000 initiating a DMARD. Forward is a large longitudinal rheumatic disease registry in the US. RA patients’ diagnoses were rheumatologist-confirmed, and every 6 months participants completed comprehensive questionnaires regarding symptoms, disease outcomes, medications, and clinical events. Lung disease (LD+) was defined as at least one of the following: emphysema, asthma, bronchitis, COPD, pleural effusion, fibrosis of the lung, “RA lung”, or ILD, the later classified by ICD9 codes (England 2019). DMARDs were categorized hierarchically into four groups: csDMARDs, TNFi and NTNFi (bDMARDs), and tsDMARDs. Percentage of patients who initiated different DMARDs were reported for pts with LD+/LD-. Discontinuation was analyzed by Kaplan Meier (KM) curves, log-ranks tests, and Cox regression models using time-varying covariates. Best models were created using backward selection models (10% probability of removal) and pre-defined clinical models.Results:Of the 21,525 eligible RA patients, 13.8% had LD+ at the time they initiated a DMARD (follow-up: 69,597 pt-yrs (median 1.9 yrs/pt)). LD+ patients tended to have more severe RA outcomes and comorbidities. MTX-monotherapy (48% vs 44%, p<0.001) and NTNFi were initiated more frequently in LD+ pts with lower use of TNFi (Figure). DMARD discontinuation rates were higher among LD+ patients for all DMARD groups, but KM curves were only significantly different for csDMARDs and TNFi. Different HRs for LD+ were found depending on the model used ranging from 1.18 to 1.28, and all models revealed an increased risk of discontinuation for LD+ patients. Compared to csDMARDs, TNFi were more often discontinued (Table). Other variables associated with an increased risk of discontinuation included: HAQ, Rheumatoid Disease (RD) comorbidity index, pain, prior bDMARDs, and csDMARDs.Conclusion:Different DMARD treatment patterns were found for LD+ patients, who tended to initiate more csDMARD and NTNFi and less likely to initiate a TNFi. LD+ patients were at a higher risk of discontinuation irrespectively of the DMARD treatment, but with greater risk for TNF users.References:[1]England BR, et al. Arth Care Res. doi:10.1002/acr.24043.Figure.DMARD treatment initiators by disease groupTable .Cox models for DMARD discontinuation by stepwise (removal probability 10%) and clinical models including DMARD treatment.Model of DMARD persistence*Model 1- Stepwise-Without drugsModel 2 – StepwiseModel 3 - ClinicalLD+ vs LD–1.181.281.20(1.08 - 1.29)(1.13 - 1.45)(1.08 - 1.34)TNF vs csDmard1.321.22(1.08 - 1.63)(1.04 - 1.44)NTNF vs csDmard1.131.13(0.83 - 1.52)(0.90 - 1.41)tsDmard vs csDmard1.301.02(0.65 - 2.60)(0.64 - 1.62)*Best models searched/Clinical adjusted for LD+/LD-, DMARDs, age, sex, education, HAQ disability, RD comorbidity index, smoking, pain, glucocorticoids, year of entry, prior bDMARDs and csDMARDs counts and MRC breath scale.Disclosure of Interests:Sofia Pedro: None declared, Ted Mikuls Grant/research support from: Horizon Therapeutics, BMS, Consultant of: Pfizer, Joe Zhuo Shareholder of: Bristol-Myers Squibb, Employee of: Bristol-Myers Squibb, Kaleb Michaud: None declared

Pulmonary Manifestations in Rheumatoid Arthritis, Psoriatic Arthritis and Peripheral Spondyloarthritis Prevalence, Diagnostic Approach and Treatment Options

2020 ◽  
Vol 16 ◽  
Author(s):  
Daniel Dejcman ◽  
Valentin Sebastian Schäfer ◽  
Dirk Skowasch ◽  
Carmen Pizarro ◽  
Andreas Krause ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Psoriatic Arthritis ◽  
Lung Disease ◽  
Current Knowledge ◽  
Treatment Options ◽  
Pulmonary Diseases ◽  
Chronic Obstructive ◽  
Pulmonary Manifestations ◽  
Major Cluster ◽  
Peripheral Spondyloarthritis

: Interstitial lung disease (ILD) is the most common form of pulmonary impairment in patients with rheumatoid arthritis (RA). However, patients with RA or other arthritic diseases such as psoriatic arthritis (PsA) or peripheral spondyloarthritis (pSpA) may develop several other pulmonary diseases such as chronic obstructive lung disease (COPD) with a higher risk than patients without arthritis. The article at hand aims at summarizing the current knowledge on the prevalence of pulmonary diseases in the above-mentioned forms of arthritis, the challenges for prevalence studies and detecting pulmonary diseases in patients with arthritis as well as possible treatment options. Dyspnea, cough or other pulmonary symptoms or findings in arthritis patients should prompt gradual diagnostic procedures considering pulmonary manifestations as a major cluster of differential diagnosis. Considering its poor prognosis and morbidity burden, RA-ILD needs to be ruled out. Treatment of manifestations often lacks solid evidencebased guidelines and referrals to specialized centers are often necessary.

scholarly journals Fast-Onset Diffuse Interstitial Lung Disease in Anti-MDA5 Antibodies-Associated Amyopathic Dermatomyositis

2021 ◽  
Vol 11 (2) ◽  
pp. 235-240
Author(s):  
Houari Aissaoui ◽  
Kinan Drak Alsibai ◽  
Naji Khayath
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Distress Syndrome ◽  
Early Stage ◽  
Immunosuppressive Treatment ◽  
Amyopathic Dermatomyositis ◽  
Pulmonary Manifestations ◽  
Life Threatening ◽  
Adequate Management ◽  
Chest Ct Scan

Anti-MDA5 antibodies-associated amyopathic dermatomyositisis a rare autoimmune disease that involve polyarthritis, cutaneous and pulmonary manifestations. The development of rapidly progressing interstitial lung disease is a life-threatening complication. We report the case of a 45-year-old woman without medical history, who was addressed to the Pulmonary Department for a polyarthritis with dry cough and hypoxemic dyspnea. Initially there was neither cutaneous manifestation nor interstitial lung disease on chest CT scan. After a few days, the patient developed fatal acute respiratory failure with diffuse ground glass opacities. Identification of anti-MDA5 antibodies allowed establishing diagnosis, despite the fact that the first immunological assessment was negative. Corticosteroid bolus of 1 g for three days and immunosuppressive treatment by cyclophosphamide was only initiated at the acute respiratory distress syndrome stage. Given the rapidly unfavorable prognosis of this entity of amyopathic dermatomyositis, the testing for anti-MDA5 antibodies should be recommended in case of progressive pulmonary symptoms associated with joint signs in order to identify this disease at an early stage and to begin rapid and adequate management.

scholarly journals Extracellular amoebal-vesicles: potential transmission vehicles for respiratory viruses

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Rafik Dey ◽  
Melanie A. Folkins ◽  
Nicholas J. Ashbolt
Keyword(s):  
Respiratory Syncytial Virus ◽  
Respiratory Tract ◽  
Respiratory Tract Infections ◽  
Respiratory Viruses ◽  
Environmental Persistence ◽  
Viral Dissemination ◽  
Acute Respiratory Tract Infections ◽  
High Concentrations ◽  
Syncytial Virus ◽  
Tract Infections

AbstractHuman respiratory syncytial virus (RSV) is a major cause of acute respiratory tract infections in children and immunocompromised adults worldwide. Here we report that amoebae-release respirable-sized vesicles containing high concentrations of infectious RSV that persisted for the duration of the experiment. Given the ubiquity of amoebae in moist environments, our results suggest that extracellular amoebal-vesicles could contribute to the environmental persistence of respiratory viruses, including potential resistance to disinfection processes and thereby offering novel pathways for viral dissemination and transmission.

scholarly journals The lung microbiota in Korean patients with non-tuberculous mycobacterial pulmonary disease

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Sung-Yoon Kang ◽  
Hyojung Kim ◽  
Sungwon Jung ◽  
Sang Min Lee ◽  
Sang Pyo Lee
Keyword(s):  
Principal Component Analysis ◽  
Respiratory Tract ◽  
Pulmonary Disease ◽  
Beta Diversity ◽  
Lower Respiratory Tract ◽  
Principal Component ◽  
Control Group ◽  
Flexible Bronchoscope ◽  
Bronchial Washing

Abstract Background The microbiota of the lower respiratory tract in patients with non-tuberculous mycobacterial pulmonary disease (NTM-PD) has not been fully evaluated. We explored the role of the lung microbiota in NTM-PD by analyzing protected specimen brushing (PSB) and bronchial washing samples from patients with NTM-PD obtained using a flexible bronchoscope. Results Bronchial washing and PSB samples from the NTM-PD group tended to have fewer OTUs and lower Chao1 richness values compared with those from the control group. In both bronchial washing and PSB samples, beta diversity was significantly lower in the NTM-PD group than in the control group (P = 2.25E-6 and P = 4.13E-4, respectively). Principal component analysis showed that the PSBs and bronchial washings exhibited similar patterns within each group but differed between the two groups. The volcano plots indicated differences in several phyla and genera between the two groups. Conclusions The lower respiratory tract of patients with NTM-PD has a unique microbiota distribution that is low in richness/diversity.

scholarly journals FRI0056 LUNG COMPROMISE SCREENING IN PATIENTS WITH EARLY RA. A MULTICENTRIC CROSS SECTIONAL STUDY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 604.1-604
Author(s):  
F. Benavidez ◽  
G. Rodriguez ◽  
A. Riopedre ◽  
D. Mata ◽  
A. Benitez ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Physical Examination ◽  
Lung Disease ◽  
Lung Involvement ◽  
High Resolution Computed Tomography ◽  
Cross Sectional ◽  
Multicentric Study ◽  
Link Type ◽  
Immunological Tests ◽  
Pulmonary Manifestations

Background:Rheumatoid arthritis (RA) affects 0.4-1.3% of general population (1). It can affect lungs in different ways, with interstitial lung disease (ILD) as the most severe. Clinically evident ILD has been reported in 10-42% of patients, with a great impact in prognosis (2).Objectives:Toidentify the prevalence of lung involvement in early rheumatoid arthritis patients (ERA) without previous known lung disease and describe the association between high resolution computed tomography (HRCT), lung functional tests (LFT) and clinical findings.Methods:Cross sectional multicentric study. We included ERA patients (1 year or less since diagnose) consecutively. Patients with previous RA related lung disease or biologic/targeted synthetic Dmard treatment were excluded. HRCT, immunological tests (rheumatoid factor, anti-CCP, ANA), LFT and clinical evaluation were performed.Results:We included 74 patients, 63 (85,1%) woman, mean (SD) of 47 (17,7) years. Thirty-seven patients (50%) were current or former smokers. Abnormal findings in HRCT were found in 62 patients (88,6%): ILD in 6 (8,6%), airway involvement in 40 (70%) and emphysema in 7 (10%). Ten patients (13,5%) had abnormal auscultation (2 sibilances, 2 roncus, and 6 crackles). Six patients (8,1%) had digital clubbing. Regarding immunological tests, 54/61 (88,5%) patients were positive for Anti CCP, and 53/61 (86,9%) were positive for FR. We compared features of patients with findings related to RA in HRCT (interstitial and/or airway) with those without them. We found no differences in the mean (SD) of DAS-28 [4,74 (1,38) vs 4,32 (1,39); p= 0,27]. The prevalence of anti- CCP was not higher in patients with abnormal HRCT [38/44 (86,3%) vs 16/17 (94,1%); p=0,39]. Patients with abnormal HRCT were older [median (IQR) 50,5 years (44,5-59,5) vs 43 years (32-51); p=0,008) and showed higher VSG values [mean (SD) 39,09 (24,03) vs 27,38 (17,6); p= 0,043]. Abnormal physical examination or dyspnea (class 2 mMRC or higher) was significantly associated with HRCT abnormalities [26 (50%) vs 3 (13,6%); p=0.003) and the presence of ILD on HRCT was significantly associated with crackles on the auscultation [4/68(6,25%) vs 2/6 (33,33%); p 0,023].Conclusion:This study shows a high prevalence of lung involvement in ERA patients of less 1 year from diagnosis. Also, we showed a significant association between HRCT and physical examination findings. This data highlights the importance of the clinical examination in Rheumatoid Arthritis patients. More studies with bigger samples and longitudinal follow up are needed to confirm and complete our results.References:[1]Rooney BK, Silman AJ. Epidemiology of the rheumatic diseases. Curr Opin Rheumatol [Internet]. 1999 Mar [cited 2016 Jul 19];11(2):91–7. Available from:http://www.ncbi.nlm.nih.gov/pubmed/10319210.[2]Antin-Ozerkis D, Evans J, Rubinowitz A, Homer RJ, Matthay RA. Pulmonary Manifestations of Rheumatoid Arthritis. Clin Chest Med [Internet]. 2010;31(3):451–78. Available from:http://dx.doi.org/10.1016/j.ccm.2010.04.003.Disclosure of Interests:None declared

scholarly journals EP06 COVID-19 masquerading as myositis and myopericarditis

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Alexa José Escudero Siosi ◽  
Hudaifa Al Ani ◽  
Antoni Chan
Keyword(s):  
Case Report ◽  
Chest Pain ◽  
Pericardial Effusion ◽  
Respiratory Tract ◽  
Medical History ◽  
Muscle Weakness ◽  
Cardiac Involvement ◽  
Sudden Onset ◽  
Pulmonary Manifestations ◽  
Rare Manifestation

Abstract Case report - Introduction Coronavirus (SARS-COV-19) typically targets the respiratory tract; however extra-respiratory manifestations such as myositis and myopericarditis may be the only presenting feature. We present a patient with myopericarditis who developed sudden onset muscle weakness. CT thorax showed typical appearance of COVID-19 with an absence of respiratory symptoms. MRI of both thighs revealed diffuse symmetrical myositis. Her clinical and paraclinical abnormalities improved with the aid of steroids. We present our approach to the case and highlight that clinicians should consider myositis as another COVID-19 manifestation when reviewing the differentials. Case report - Case description A 50-year-old female, non-smoker, presented with few days history of central chest pain radiating to her back. This was exacerbated by lying down and inspiration. Associated with mild shortness of breath on exertion. She denied upper respiratory tract symptoms. Her past medical history included hypertension and myopericarditis in 2012 and 2013 requiring pericardiocentesis. In 2017 she presented with post-streptococcal erythema nodosum and reactive arthritis in left ankle. On auscultation her heart sounds were normal, and chest was clear. Initial investigations revealed a mild lymphopenia 0.63, a C-reactive protein of 11mg/L, and a raised troponin 77 and 103 on repeat. D-dimer, Chest x-ray were normal. ECHO showed trivial anterior pericardial effusion, good biventricular function. Treatment included colchicine 500 micrograms four times a day and Ibuprofen 400 mg three times a day. On her second day of admission she developed hypotensive episodes BP 75/49 mm/Hg and mild pyrexia of 37.5 degrees. Her chest pain continued. Electrocardiogram was normal, repeat echocardiogram showed stable 1.40 cm pericardial effusion. CT thorax revealed no dissection or features suggesting pulmonary sarcoidosis but ground-glass opacity changes in keeping with COVID-19. Her COVID-19 swab test came back positive. On the 4th day of admission, she complained of sudden onset of severe pain affecting her thighs, shoulders, and arms, with marked proximal lower limbs and truncal weakness. Because of this, she struggled to mobilise. There was a rapid rise in her creatine kinase from 6.423U/L (day 5) to 32.230 U/L (day 7). ALT increased to 136. MRI showed diffuse myositis with symmetrical appearances involving the anterior, medial, and posterior muscle compartments of both thighs. In view of her previous and current presentation, autoimmune screen and extended myositis immunoblot were sent and were negative. Interestingly, her clinical and paraclinical abnormalities improved dramatically after few days with no steroids initially. Case report - Discussion The identification of extra-pulmonary manifestations neurological, cardiac, and muscular have recently increased as the number of COVID-19 cases grow. This case highlights cardiac and skeletal muscle involvement could perhaps represent early or only manifestation of COVID-19. Cardiac involvement in COVID-19 commonly manifests as acute cardiac injury (8–12%), arrhythmia (8.9–16.7%) and myocarditis. In our case the cardiac MRI demonstrated evidence of myocarditis in the basal inferoseptum and apex. Myalgia and muscle weakness are among the symptoms described by patients affected by COVID-19. Some studies report the prevalence of myalgia to be between 11%-50%. The onset of symptoms and the fact that her symptoms improved rapidly led us to consider a viral myositis as the underlying cause, the viral component being COVID-19. We also considered other potential causes. There are reported cases of colchicine myopathy however this is more common in patients with renal impairment, which was absent in this case. On further examination she did not have other clinical signs or symptoms of connective tissue disease or extra muscular manifestation of autoimmune myositis. Her abnormal ALT may be derived from damaged muscle, and therefore in this context is not necessarily a specific indicator of liver disease. Interestingly abnormal liver function tests have been attributed in 16 - 53% of COVID-19 cases. Little is known about the multiple biologic characteristics of COVID-19 and there are no established clinic serological criteria for COVID-19 related myositis nor useful values/cut offs to exclude cardiac involvement in myositis, further research is therefore warranted. In conclusion, clinicians should be aware of the rare manifestation of COVID-19 and consider this in the differentials. Of course, it is important in the first instant to rule out any serious underlying disease or overlap disorder before attributing symptoms to COVID-19. Case report - Key learning points  Myositis is a rare manifestation of COVID-19 that clinicians should be aware of.Detailed medical history, examination and investigations identifies the most likely underlying cause.In the right clinical context, COVID-19 – 19 testing should be included in baseline tests of patients presenting with myositis.

THE RELATIONSHIP OF AMNIOTIC FLUID VISCOSITY TO RESPIRATORY DISTRESS IN LAMBS

PEDIATRICS ◽  
1966 ◽  
Vol 38 (5) ◽  
pp. 858-864
Author(s):  
Arthur J. Moss ◽  
Ovidio Rettori ◽  
Norman S. Simmons
Keyword(s):  
Cesarean Section ◽  
Respiratory Distress ◽  
Amniotic Fluid ◽  
Fetal Distress ◽  
In Utero ◽  
Fluid Viscosity ◽  
Lung Expansion ◽  
Relationship Of ◽  
The Relationship ◽  
Length Of Gestation

The viscosity of amniotic fluid was measured in 52 ewes and the results correlated with the postnatal course of the lambs delivered by cesarean section. Viscosity was not related to length of gestation or to the immediate prepartum condition of the ewe, but a definite relationship was found between amniotic fluid viscosity (AFV) and the postnatal course of the fetus. Of 22 lambs considered viable, 9 failed to survive. In 10 of the 13 survivors, AFV was less than 1.6; whereas in the nonsurvivors, AFV varied between 1.6 and 4.4. All of the nonsurvivors experienced respiratory difficulty associated with copious amounts of extremely viscous secretions in the mouth and oropharynx. The substance responsible for the high AFV was a mucoprotein and was detectable in all samples with a viscosity of 1.24 or more but in none with a viscosity below 1.24. The data suggest the possibility that fetal distress may induce qualitative or quantitative alterations of fetal mucous secretions in utero which subsequently could impede lung expansion at birth.

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