scholarly journals A Case of Dermatomyositis and Anti-EJ Autoantibody with Chronic Intestinal Pseudoobstruction Successfully Treated with Octreotide

2016 ◽  
Vol 2016 ◽  
pp. 1-5
Author(s):  
Chiho Yamada ◽  
Shinji Sato ◽  
Noriko Sasaki ◽  
Takayoshi Kurabayashi ◽  
Sho Sasaki ◽  
...  
Keyword(s):  
Abdominal Pain ◽  
Connective Tissue ◽  
Medical Treatment ◽  
Connective Tissue Disease ◽  
Intravenous Administration ◽  
Subcutaneous Injection ◽  
Intestinal Pseudoobstruction ◽  
Chronic Intestinal Pseudoobstruction ◽  
Life Threatening ◽  
Long Acting

Chronic intestinal pseudoobstruction (CIPO) is a serious complication in patients with connective tissue disease (CTD) and is sometimes life-threatening or fatal despite intensive medical treatment. Here, we report a patient with dermatomyositis (DM) and anti-EJ autoantibody who developed CIPO that was improved by octreotide. Because her abdominal pain and bloatedness were so severe and persistent, we introduced octreotide to relieve symptoms. In this case, continuous intravenous administration as well as long-acting subcutaneous injection of octreotide was effective for treating CIPO.

scholarly journals Kingella kingae Intrauterine Infection: An Unusual Cause of Chorioamnionitis and Miscarriage in a Patient with Undifferentiated Connective Tissue Disease

Diagnostics ◽  
2021 ◽  
Vol 11 (2) ◽  
pp. 243
Author(s):  
Maria Paola Bonasoni ◽  
Andrea Palicelli ◽  
Giulia Dalla Dea ◽  
Giuseppina Comitini ◽  
Giulia Pazzola ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Medical Treatment ◽  
Connective Tissue Disease ◽  
Lupus Erythematosus ◽  
Etiologic Agent ◽  
Intrauterine Fetal Death ◽  
Severe Neutropenia ◽  
Invasive Infection ◽  
Undifferentiated Connective Tissue Disease ◽  
Kingella Kingae

Kingella kingae is a Gram-negative coccobacillus belonging to the Neisseriaceae family. In children less than 4 years old, K. kingae invasive infection can induce septic arthritis and osteomyelitis, and more rarely endocarditis, meningitis, ocular infections, and pneumonia. In adults, it may be a cause of endocarditis. To date, K. kingae acute chorioamnionitis (AC) leading to preterm rupture of membranes (PPROM) and miscarriage has never been reported. Herein, we describe a case of intrauterine fetal death (IUFD) at 22 weeks’ gestation due to K. kingae infection occurred in a patient affected by undifferentiated connective tissue disease (UCTD) in lupus erythematosus systemic (LES) evolution with severe neutropenia. K. kingae was isolated in placental subamnionic swab and tissue cultures as well as fetal ear, nose, and pharyngeal swabs. Placental histological examination showed necrotizing AC and funisitis. In the fetus, neutrophils were observed within the alveoli and in the gastrointestinal lumen. Maternal medical treatment for UCTD was modified according to the K. kingae invasive infection. In the event of IUFD due to AC, microbiological cultures on placenta and fetal tissues should always be carried out in order to isolate the etiologic agent and target the correct medical treatment.

Life-threatening acute pneumonitis in mixed connective tissue disease: a case report and literature review

2015 ◽  
Vol 127 (19-20) ◽  
pp. 792-794 ◽  
Author(s):  
Eva Rath ◽  
Shahin Zandieh ◽  
Alexander Löckinger ◽  
Mirko Hirschl ◽  
Klaus Klaushofer ◽  
...  
Keyword(s):  
Case Report ◽  
Connective Tissue ◽  
Literature Review ◽  
Connective Tissue Disease ◽  
Mixed Connective Tissue Disease ◽  
Life Threatening

scholarly journals Octreotide treatment of chronic intestinal pseudoobstruction secondary to connective tissue diseases

1999 ◽  
Vol 42 (7) ◽  
pp. 1545-1549 ◽  
Author(s):  
Gabriel Perlemuter ◽  
Patrice Cacoub ◽  
Stanislas Chaussade ◽  
Bertrand Wechsler ◽  
Daniel Couturier ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Diseases ◽  
Intestinal Pseudoobstruction ◽  
Chronic Intestinal Pseudoobstruction ◽  
Octreotide Treatment

Microembolization from atheroembolic disease or aneurysm. A case study

1996 ◽  
Vol 86 (6) ◽  
pp. 249-252 ◽  
Author(s):  
BD Caldwell ◽  
VS Marrochello
Keyword(s):  
Connective Tissue ◽  
Early Diagnosis ◽  
Connective Tissue Disease ◽  
Initial Presentation ◽  
Vascular Pathology ◽  
Peripheral Angiography ◽  
Common Cause ◽  
Life Threatening ◽  
Blue Toe Syndrome

Cyanosis of the digits may have several etiologies ranging from trauma to connective tissue disease; however, the most common cause of the so-called blue toe syndrome is atheroembolic disease or aneurysm and is frequently misdiagnosed on initial presentation. Pedal pulses are often palpable which may misdirect the physician from a diagnosis of vascular pathology. Furthermore, the proximal source of embolic shower may be far from the sight of symptoms. Noninvasive vascular testing, peripheral angiography, abdominal and popliteal ultrasonography, and echocardiography are all techniques that may be beneficial in discovering the origin of emboli. Atheroembolisms and aneurysms can be limb-threatening or life-threatening and hence early diagnosis is imperative.

Early Diagnosis of Neutropenic Enterocolitis by Ultrasound Sonography.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4742-4742 ◽  
Author(s):  
Edoardo Benedetti ◽  
Federico Simonetti ◽  
Francesco Caracciolo ◽  
Federico Papineschi ◽  
Benedetto Bruno ◽  
...  
Keyword(s):  
Abdominal Pain ◽  
Early Diagnosis ◽  
Medical Treatment ◽  
Gram Positive ◽  
Acute Leukemias ◽  
Neutropenic Enterocolitis ◽  
Gram Negative ◽  
Life Threatening ◽  
Neutropenic Patients ◽  
Life Threatening Complication

Abstract Abstract 4742 Introduction Neutropenic enterocolitis (NEC) is a life threatening complication of chemotherapy in leukemic and solid tumor patients with an incidence ranging from 2.6% to 33%. It is a necrotizing inflammatory disease that most commonly involves the ileo-cecal region. The cecum is almost invariably affected likely due to its distensibility and limited blood supply. Macroscopically the involved bowel segments show oedematous and thickened walls, with varying degrees of ulceration and haemorrhage. Perforation occurs in 5%-10% of cases. Early diagnosis is crucial to start conservative medical treatment which appears the optimal strategy. Criteria for prompt surgical treatment have also been proposed (Shamberger RC et al, 1986) so that a careful clinical evaluation by both the physician and the surgeon is mandatory (Davila ML et al, 2004). NEC should be always suspected in neutropenic patients with abdominal pain, fever and diarrhea. Ultrasound (US) has been used to evaluate bowel-wall thickening (BWT). One study correlated BWT with clinical outcome: 60% of patients with BWT > 10 mm died from NEC as compared with 4.2% of those with BWT < 10 mm (Cartoni C. et al, 2001). Overall, despite aggressive treatment, mortality rate is up to 21-48% and patients may die within hours from the onset of acute symptoms. Because of the risk of early death, a swift diagnosis is imperative. We investigated if US could detect early signs of NEC and lead to prompt treatment. We analyzed two patients cohorts (A and B). In cohort A US was performed later in the course of the disease whereas, in cohort B US was immediately performed at the onset of a single symptom (diarrhea and/or abdominal pain whichever occurred first with/without fever). Underlying haematological diagnoses were Hodgkin Disease (N=10), acute leukemias (N=9), multiple myeloma (N=3) and non-Hodgkin lymphomas (N=10). Treatments consisted of standard chemotherapy (N=10), myeloablative allografting after busulfan/cytoxan (N=1) and cytoxan/total body irradiation (N=1); autografting after busulfan/cytoxan (N=1), BEAM (N=16) and melphalan (N=3). Results All 32 patients showed grade 4 neutropenia at the onset of symptoms. Thirty-one/32 (97%) complained of abdominal pain whereas diarrhea was present in 30/32 (94%). Positive stool (for Clostridium Difficilis and Escherichia Coli) and blood cultures (62% Gram negative and 38% Gram positive bacteria) were found in 4/32 (12.5%) and 8/32 (25%) respectively. Trans-abdominal real-time US scanning of the bowel was performed using a 3.5-5 MHz convex probe and a 7 MHz linear transducer. A portable sonographer (Esaote model My Lab 25) was used for bed-side US. Both trans-abdominal axial and transverse US scans were performed on the colon. US signs of NEC were defined as thickening or dilation of small and/or large intestine. The intestinal involved areas were ileum, 21%, last terminal ileum, 21%, cecum 7%, ascending colon, 16%, transverse colon, 16%, descending colon, 14% and jejunum, 4%. US revealed signs of NEC in 8/14 in cohort A and 18/18 in cohort B. In cohort B, early US detected signs of NEC in 7 patients with abdominal pain and diarrhea without fever. Complete response to prompt medical treatment occurred in 6. Three/7 patients developed fever 48 hours after the US-guided diagnosis of NEC. Two patients, at risk of wall rupture by US imaging (confirmed by CT scans), underwent successful colon surgery within 12 hours from diagnosis. Except for these 2, all patients received medical treatment (broad spectrum antibiotics for gram-negative, gram-positive and anaerobic coverage, and antifungal drugs, granulocyte transfusions, bowel rest and granulocyte colony stimulating factor as per internal protocol). Overall 3/32 patients died. Conclusions Early bed-side intestinal US in neutropenic patients performed at the onset of a single symptom suggestive of NEC led to timely and successful treatment of this life threatening complication even before the development of fever. Disclosures: No relevant conflicts of interest to declare.

Systemic sclerosis and related disorders

2018 ◽  
pp. 401-424
Author(s):  
Gavin Clunie ◽  
Nick Wilkinson ◽  
Elena Nikiphorou ◽  
Deepak R. Jadon
Keyword(s):  
Systemic Sclerosis ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Broad Spectrum ◽  
Clinical Manifestations ◽  
Life Threatening

This chapter focuses on systemic sclerosis, a generalized autoimmune connective tissue disease. It describes the epidemiology and classification of systemic sclerosis, along with its pathophysiology and potential triggers of disease. It also discusses the condition’s broad spectrum of clinical manifestations and highlights its potential for multiorgan involvement and life-threatening complications. Furthermore, the chapter provides insights into the definitions for related conditions such as morphoea and its different subtypes. Complexities around the treatment and a discussion on the prognosis and outcomes of systemic sclerosis and related conditions are also included.

scholarly journals Connective Tissue Disease-Associated Pulmonary Arterial Hypertension in Southern Taiwan: A Single-Center 10-Year Longitudinal Observation Cohort

Healthcare ◽  
2021 ◽  
Vol 9 (5) ◽  
pp. 615
Author(s):  
Chun-Hsin Wu ◽  
Chun-Yu Lin ◽  
Chih-Hsin Hsu ◽  
Sheng-Hsiang Lin ◽  
Chia-Tse Weng
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Connective Tissue Disease ◽  
Heart Catheterization ◽  
Mean Pulmonary Arterial Pressure ◽  
Increased Risk ◽  
Life Threatening ◽  
Pulmonary Arterial ◽  
Cox Proportional Hazard Regression

Background: Pulmonary arterial hypertension (PAH) is a life-threatening disease with different etiologies and outcomes. We aimed to explore differences in clinical features and outcomes of idiopathic PAH (iPAH) and connective tissue disease-related PAH (CTD-PAH) in Taiwanese patients and determine risk factors for mortality. Methods: We retrospectively reviewed the medical records of patients with right-sided heart catheterization-diagnosed PAH between January 2005 and December 2015. The iPAH (n = 31) and CTD-PAH (n = 14) patients were enrolled and followed until December 31, 2019. Between-group comparisons were conducted. Potential predictors of the mortality of PAH were determined using the Cox proportional hazard regression model. Results: CTD-PAH patients had higher levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) and lower predicted diffusing capacity of carbon monoxide (DLCO) than iPAH patients. The mortality rates were similar between CTD-PAH and iPAH (21.4% vs. 22.6%, p = 0.99). A mean pulmonary arterial pressure (mPAP) > 46 mmHg was a predictor of PAH-induced mortality (adjusted hazard ratio 21.8, 95% confidence interval 2.32–204.8). Conclusions: A higher mPAP level, but not underlying CTDs, imposed a significantly increased risk of mortality to patients with PAH.

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