Overlap Syndromes in Sarcoidosis: Clinical Features and Outcomes

2020 ◽  
Vol 41 (05) ◽  
pp. 607-617
Author(s):  
W. Ennis James

AbstractSarcoidosis is a multisystem inflammatory disease characterized by noncaseating granulomatous inflammation. While pulmonary sarcoidosis is most common, extrapulmonary involvement occurs in 50 to 74% of patients and can be the presenting abnormality in some patients. The diagnosis of sarcoidosis is based on a compatible clinical presentation in combination with granulomas on histology and exclusion of other causes. However, the absence of a diagnostic biomarker for sarcoidosis, in addition to the overlap of granulomatous inflammation and nonspecific clinical findings with other diseases, often results in a delayed diagnosis. Sarcoidosis overlap syndromes are typically described when sarcoidosis is diagnosed in the presence of another disease (concurrently or sequentially) with shared clinical and histologic features, or when sarcoidosis presents with clinical features typically observed in, but not diagnostic of, other diseases. Awareness of overlap syndromes is important for clinicians to avoid diagnostic errors and evaluate for concomitant diagnoses that may impact the management and outcome of sarcoidosis. This article is intended to provide an overview of these presentations and the most commonly associated diseases, with attention to their prevalence, clinical features, and reciprocal impacts on disease outcomes.

2000 ◽  
Vol 114 (4) ◽  
pp. 274-278 ◽  
Author(s):  
Muzaffer Kanlikama ◽  
Semih Mumbuç ◽  
Yildirim Bayazit ◽  
Akif S¸irikçi

The objectives of this study were to investigate the typical clinical presentation, diagnosis and treatment of mycobacterial cervical lymphadenitis (MCL).Medical records of 87 patients who were treated for MCL were retrospectively reviewed. Definitive diagnosis of MCL was made when a neck mass persisted for several weeks or months and one or more of the following was obtained: (1) positive mycobacterial cultures from biopsy material; (2) Positive mycobacterial staining of biopsy material; (3) Granulomatous inflammation and caseating necrosis on histopathological examination of biopsy material.Clinical findings were reviewed prior to treatment. The treatment included standard antituberculous medications followed by surgery in which either total excision or selective nodal dissection of the cervical lump was made. Follow-up results are presented.The chief complaint was a cervical mass that was localized mostly to the posterior cervical or submandibular regions. A fistula formation was encountered in 11.5 per cent. All patients recovered from MCL by combined antituberculous drug and surgical treatments.Clinical presentation of the disease and histopathological assessment are important in the diagnosis of MCL as well as in the differential diagnosis of tuberculous and nontuberculous MCL. Utilizing the combined medical and surgical treatment options, both tuberculous and non-tuberculous cervical adenitis can be treated successfully.


1996 ◽  
Vol 35 (01) ◽  
pp. 41-51 ◽  
Author(s):  
F. Molino ◽  
D. Furia ◽  
F. Bar ◽  
S. Battista ◽  
N. Cappello ◽  
...  

AbstractThe study reported in this paper is aimed at evaluating the effectiveness of a knowledge-based expert system (ICTERUS) in diagnosing jaundiced patients, compared with a statistical system based on probabilistic concepts (TRIAL). The performances of both systems have been evaluated using the same set of data in the same number of patients. Both systems are spin-off products of the European project Euricterus, an EC-COMACBME Project designed to document the occurrence and diagnostic value of clinical findings in the clinical presentation of jaundice in Europe, and have been developed as decision-making tools for the identification of the cause of jaundice based only on clinical information and routine investigations. Two groups of jaundiced patients were studied, including 500 (retrospective sample) and 100 (prospective sample) subjects, respectively. All patients were independently submitted to both decision-support tools. The input of both systems was the data set agreed within the Euricterus Project. The performances of both systems were evaluated with respect to the reference diagnoses provided by experts on the basis of the full clinical documentation. Results indicate that both systems are clinically reliable, although the diagnostic prediction provided by the knowledge-based approach is slightly better.


2021 ◽  
Vol 29 (1) ◽  
pp. 230949902199399
Author(s):  
Taketsugu Fujibuchi ◽  
Hiroshi Imai ◽  
Joji Miyawaki ◽  
Teruki Kidani ◽  
Hiroshi Kiyomatsu ◽  
...  

Purpose: The spectrum of diagnoses and clinical features of hand tumors differ from those of tumors in other body parts. However, only a few reports have comprehensively referenced the diagnosis and clinical features of hand tumors. This study aimed to elucidate the diagnostic distribution and the clinical features of hand tumors undergone surgery in our institute. Patients and methods: A total of 235 lesions in 186 patients diagnosed with hand tumors between 1978 and 2020 were reviewed. Age at surgery, gender, chief complaint, tumor location, and pathological diagnosis were analyzed. Results: There were 121 benign bone tumors, 98 benign soft tissue tumors, and 16 malignant tumors. Chondroma and tenosynovial giant cell tumor were common benign bone and soft tissue tumors at the proximal phalanx of the ring finger and the palm, respectively. Meanwhile, chondrosarcoma and synovial sarcoma were common malignant tumors at the dorsal part of the hand. Local pain and painless mass were the chief complaints in patients with benign bone and soft tissue tumors, respectively. Most patients with malignant tumors were referred after unplanned resection. When patients were classified into two categories by tumor size according to maximal diameter, tumors larger than 19 mm had a significantly higher risk of malignant ( p = 0.031) despite being smaller than other tumors in different body parts. Conclusion: When a tumor malignancy is suspected, the patient should be referred to a specialist to avoid unplanned resection or delayed diagnosis due to misdiagnosis. Knowing the distribution and clinical features should help in diagnosing hand tumors.


2021 ◽  
pp. 1-12
Author(s):  
Zongqiong Sun ◽  
Linfang Jin ◽  
Shuai Zhang ◽  
Shaofeng Duan ◽  
Wei Xing ◽  
...  

PURPOSE: To investigate feasibility of predicting Lauren type of gastric cancer based on CT radiomics nomogram before operation. MATERIALS AND METHODS: The clinical data and pre-treatment CT images of 300 gastric cancer patients with Lauren intestinal or diffuse type confirmed by postoperative pathology were retrospectively analyzed, who were randomly divided into training set and testing set with a ratio of 2:1. Clinical features were compared between the two Lauren types in the training set and testing set, respectively. Gastric tumors on CT images were manually segmented using ITK-SNAP software, and radiomic features of the segmented tumors were extracted, filtered and minimized using the least absolute shrinkage and selection operator (LASSO) regression to select optimal features and develop radiomics signature. A nomogram was constructed with radiomic features and clinical characteristics to predict Lauren type of gastric cancer. Clinical model, radiomics signature model, and the nomogram model were compared using the receiver operating characteristic (ROC) curve analysis with area under the curve (AUC). The calibration curve was used to test the agreement between prediction probability and actual clinical findings, and the decision curve was performed to assess the clinical usage of the nomogram model. RESULTS: In clinical features, Lauren type of gastric cancer relate to age and CT-N stage of patients (all p <  0.05). Radiomics signature was developed with the retained 10 radiomic features. The nomogram was constructed with the 2 clinical features and radiomics signature. Among 3 prediction models, performance of the nomogram was the best in predicting Lauren type of gastric cancer, with the respective AUC, accuracy, sensitivity and specificity of 0.864, 78.0%, 90.0%, 70.0%in the testing set. In addition, the calibration curve showed a good agreement between prediction probability and actual clinical findings (p >  0.05). CONCLUSION: The nomogram combining radiomics signature and clinical features is a useful tool with the increased value to predict Lauren type of gastric cancer.


2021 ◽  
Vol 8 (4) ◽  
Author(s):  
Saeed Shoar ◽  
Fernando H Centeno ◽  
Daniel M Musher

Abstract Background Long regarded as the second most common cause of community-acquired pneumonia (CAP), Haemophilus influenzae has recently been identified with almost equal frequency as pneumococcus in patients hospitalized for CAP. The literature lacks a detailed description of the presentation, clinical features, laboratory and radiologic findings, and outcomes in Haemophilus pneumonia. Methods During 2 prospective studies of patients hospitalized for CAP, we identified 33 patients with Haemophilus pneumonia. In order to provide context, we compared clinical findings in these patients with findings in 36 patients with pneumococcal pneumonia identified during the same period. We included and analyzed separately data from patients with viral coinfection. Patients with coinfection by other bacteria were excluded. Results Haemophilus pneumonia occurred in older adults who had underlying chronic lung disease, cardiac conditions, and alcohol use disorder, the same population at risk for pneumococcal pneumonia. However, in contrast to pneumococcal pneumonia, patients with Haemophilus pneumonia had less severe infection as shown by absence of septic shock on admission, less confusion, fewer cases of leukopenia or extreme leukocytosis, and no deaths at 30 days. Viral coinfection greatly increased the severity of Haemophilus, but not pneumococcal pneumonia. Conclusions We present the first thorough description of Haemophilus pneumonia, show that it is less severe than pneumococcal pneumonia, and document that viral coinfection greatly increases its severity. These distinctions are lost when the label CAP is liberally applied to all patients who come to the hospital from the community for pneumonia.


2021 ◽  
Vol 10 (11) ◽  
pp. 2439
Author(s):  
Jerome R. Lechien ◽  
Stéphane Hans ◽  
Francois Bobin ◽  
Christian Calvo-Henriquez ◽  
Sven Saussez ◽  
...  

Background: Laryngopharyngeal reflux (LPR) is a common disease in otolaryngology characterized by an inflammatory reaction of the mucosa of the upper aerodigestive tract caused by digestive refluxate enzymes. LPR has been identified as the etiological or favoring factor of laryngeal, oral, sinonasal, or otological diseases. In this case series, we reported the atypical clinical presentation of LPR in patients presenting in our clinic with reflux. Methods: A retrospective medical chart review of 351 patients with LPR treated in the European Reflux Clinic in Brussels, Poitiers and Paris was performed. In order to be included, patients had to report an atypical clinical presentation of LPR, consisting of symptoms or findings that are not described in the reflux symptom score and reflux sign assessment. The LPR diagnosis was confirmed with a 24 h hypopharyngeal-esophageal impedance pH study, and patients were treated with a combination of diet, proton pump inhibitors, and alginates. The atypical symptoms or findings had to be resolved from pre- to posttreatment. Results: From 2017 to 2021, 21 patients with atypical LPR were treated in our center. The clinical presentation consisted of recurrent aphthosis or burning mouth (N = 9), recurrent burps and abdominal disorders (N = 2), posterior nasal obstruction (N = 2), recurrent acute suppurative otitis media (N = 2), severe vocal fold dysplasia (N = 2), and recurrent acute rhinopharyngitis (N = 1), tearing (N = 1), aspirations (N = 1), or tracheobronchitis (N = 1). Abnormal upper aerodigestive tract reflux events were identified in all of these patients. Atypical clinical findings resolved and did not recur after an adequate antireflux treatment. Conclusion: LPR may present with various clinical presentations, including mouth, eye, tracheobronchial, nasal, or laryngeal findings, which may all regress with adequate treatment. Future studies are needed to better specify the relationship between LPR and these atypical findings through analyses identifying gastroduodenal enzymes in the inflamed tissue.


2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Yu-Liang Jiang ◽  
Xiao-Dong Xu ◽  
Bai-Rong Li ◽  
En-Da Yu ◽  
Zi-Ye Zhao ◽  
...  

Abstract Objective To report Peutz–Jeghers syndrome (PJS) cases with non-definitive clues in the family or personal history and finally diagnosed through pathological examination and STK11 gene mutation test. Clinical presentation and intervention PJS was suspected in 3 families with tortuous medical courses. Two of them had relatives departed due to polyposis or colon cancer without pathological results, and the other one had been diagnosed as hyperplastic polyposis before. Diagnosis of PJS was confirmed by endoscopy and repeated pathological examinations, and the STK11 mutation test finally confirmed the diagnosis at genetic level, during which 3 novel mutation were detected (536C > A, 373_374insA, 454_455insGGAGAAGCGTTTCCCAGTGTGCC). Conclusion Early diagnosis of PJS is important and may be based on a family history with selective features among family members, and the pathological information is the key. The novel mutations also expand the STK11 variant spectrum.


Diagnostics ◽  
2021 ◽  
Vol 11 (4) ◽  
pp. 680
Author(s):  
Takashi Kojima ◽  
Murat Dogru ◽  
Eisuke Shimizu ◽  
Hiroyuki Yazu ◽  
Aya Takahashi ◽  
...  

Granulomatosis with polyangiitis (GPA) presents with a variety of systemic findings, sometimes with ocular findings initially, but is often difficult to diagnose at an early stage. An 85-year-old male had complaints of ocular dryness and redness and was diagnosed with meibomian gland dysfunction with meibomitis. Despite an initial treatment with topical steroid and antibiotics, the meibomitis did not improve and the left eye developed scleritis and iridocyclitis. The patient was administered topical mydriatics and oral steroids. During follow-up, the patient developed left hearing difficulty and reported a darker urine. Urinalysis revealed microscopic hematuria. A blood test showed an elevated erythrocyte sedimentation rate, positivity for perinuclear anti-neutorophil cytoplasmic antibody, and elevations in blood urea nitrogen and serum creatinine. Nasal mucosal biopsy showed a non-necrotizing granulomatous inflammation. Renal biopsy revealed focal glomerulosclerosis. Cystoscopy and bladder wash followed by a planned transurethral resection revealed atypical cells and apical papillary tumors which were resected. Iridocyclitis and scleritis responded well to oral prednisolone with 0.1% topical betamethasone and prednisolone ointment. The patient is tumor free with no recurrences 24 months after resection. GPA may present atypically with meibomian gland dysfunction without showing representative clinical findings. Early detection and treatment are essential for visual recovery.


2018 ◽  
Author(s):  
V Kouranos ◽  
S Ward ◽  
M Kokosi ◽  
D Castillo ◽  
F Chua ◽  
...  

2010 ◽  
Vol 2010 ◽  
pp. 1-4 ◽  
Author(s):  
Ibrahim Altraif ◽  
Fayaz A. Handoo ◽  
Khaled O. Alsaad ◽  
Adel Gublan

Although systemic amyloidosis of amyloid-associated protein (AA) type (secondary or reactive amyloidosis) frequently involves the liver, it rarely causes clinically apparent liver disease. Mild elevation of alkaline phosphatase and hepatomegaly are the most common biochemical and clinical findings, respectively. We report a case of systemic amyloidosis of AA type, which clinically presented as subacute hepatic failure and resulted in a fatal clinical course in a 69-year-old man. To the best of our knowledge, this is the fifth case of hepatic amyloidosis of AA type that clinically presented as fatal subacute hepatic failure, an unusual clinical presentation for hepatic involvement by systemic AA-type amyloid.


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