Faculty Opinions recommendation of Histopathology of explanted lungs from patients with a diagnosis of pulmonary sarcoidosis.

Author(s):  
Marc Judson
2010 ◽  
Vol 30 (03) ◽  
pp. 156-161 ◽  
Author(s):  
R. Gheisari ◽  
B. Bomke ◽  
T. Hoffmann ◽  
R. E. Scharf

SummaryWe have performed a monocenter study on 29 consecutive patients with acquired haemophilia A who were referred for diagnosis and treatment to the Düsseldorf Haemophilia Comprehensive Care Center between March 2001 and February 2010. Patients, methods: 18 men (age: 44–86 years) and 11 women (age: 20–83 years). For laboratory evaluation, a standardized staged protocol of aPTT, FVIII : C activity and concentration, mixing studies with patient and normal plasma, and quantification of inhibitor titers (Bethesda assay) was used. Diagnostic work-up included elaborate examinations for any underlying disease. Results: In 18 (62%) of the 29 patients with acquired haemophilia A, an underlying disorder was identified, including 9 patients with respiratory diseases (31%), 7 patients with autoimmune disorders (24%), one with malignancy, and one with postpartum state, while in 11 patients (38%) acquired haemophilia A remained idiopathic. Haemotherapy of bleeding, suppression or elimination of the inhibitor, and induction of immunotolerance to endogenous FVIII:C were performed according to a treatment algorithm. Predefined clinical endpoints were control of bleeding, eradication of the inhibitor, complete or partial remission (CR, PR), relapse, or early death (≤30 days). Of the 29 patients in total, 22 individuals achieved CR (76%), three had PR, one relapsed, and three died within 30 days (one of acute myocardial infarction while on anti-haemorrhagic treatment, one of sepsis while on immunosuppression due to active acquired haemophilia A, one of lung bleeding in association with pre-existing pulmonary sarcoidosis). Conclusion: This monocenter study demonstrates that control of life-threatening bleeding, eradication of the inhibitor, and induction of tolerance to endogenous FVIII have significantly improved the clinical outcome of acquired haemophilia A. Our data also suggest a shift in underlying disorders associated with acquired haemophilia A, whereby, in comparison to published studies, a relative increase in the proportion of patients with respiratory diseases is present.


Author(s):  
Lutfullah Sari ◽  
Abdusselim Adil Peker ◽  
Dilek Hacer Cesme ◽  
Alpay Alkan

Background: Neurosarcoidosis manifests symptomatically in 5% of patients with sarcoidosis and diagnosis can be challenging if not clinically suspected. Cerebral mass-like presentation of neurosarcoidosis rarely reported in the literature. We presented a woman with neurosarcoidosis who had a cerebral mass-like lesion which completely disappeared after medical treatment. Discussion: A 37-year-old woman with history of pulmonary sarcoidosis referred to the emergency service of our hospital with a one-month history of progressive dizziness, nausea and seeing flashing lights. At neurologic examination, numbness and weakness on the left side of the body, deviation of uvula toward the right side was seen. Cranial MRI demonstrated a 2.5x2 cm in size mass lesion which hypointense on T1 WI, heterogeneous hyperintense on T2 and FLAIR sequence with peripheral vasogenic edema and heterogeneous, irregular contrast enhancement simulating brain tumor. Also, leptomeningeal and nodular contrast enhancement was seen on brainstem, cerebellar vermis, perimesencephalic cistern and left frontal, bilateral parietooccipital sulcus. In laboratory tests; The level of serum angiotensin-converting enzyme (ACE) was 53 IU/mL (N:8-52 IU/mL) and cerebrospinal fluid (CSF) ACE was 23 IU/mL (N:0-2.6 IU/mL). CSF cytology analysis was normal. Pattern 2 oligoclonal bands were present. With these clinical, laboratory and radiological findings, cerebral involvement of sarcoidosis was suspected. Biopsy was not performed due to the high risk of morbidity caused by the deep location of the lesion.Patient was treated with methylprednisolone and Azathioprine for a month.On post-treatment control imaging; lesion disappeared completely without residual leptomeningeal and nodular contrast enhancement.Also, neurologic symptoms were decreased remarkably. Conclusion: Multi-system inflammatory disorders like sarcoidosis, can present with mass-like lesion in the brain parenchyma. While early diagnosis is important to prevent unnecessary interventions like biopsy and surgery, it is crucial to initiate the necessary treatment with the aim of recovery without sequelae. Radiological and clinical follow-up are fundamental in differential diagnosis.


Author(s):  
Marco Tana ◽  
Silvio di Carlo ◽  
Marcello Romano ◽  
Massimo Alessandri ◽  
Cosima Schiavone ◽  
...  

Background:18F-fluorodeoxyglucose positron emission tomography integrated with computed tomography (18-F-FDG-PET/CT) is getting wide consensus in the diagnosis and staging of neoplastic disorders and represents a useful tool in the assessment of various inflammatory conditions. </P><P> Discussion: Sarcoidosis is an uncommon disease characterized by the systemic formation of noncaseating granulomas. Lungs are the sites most often affected, and investigation with high resolution computed tomography and biopsy is essential to achieve a correct diagnosis. 18-F-FDGPET/ CT is effective in the assessment of pulmonary sarcoidosis by demonstrating pulmonary and extrathoracic involvement and findings correlate well with pulmonary function in patients affected.Conclusion:This review would illustrate the usefulness and limits of 18-F-FDG-PET/CT in the assessment of pulmonary sarcoidosis.


CHEST Journal ◽  
2020 ◽  
Vol 157 (6) ◽  
pp. A216
Author(s):  
S. Papiris ◽  
E. Manali ◽  
N. Pianou ◽  
M. Kallergi ◽  
A. Papaioannou ◽  
...  

Thorax ◽  
2001 ◽  
Vol 56 (6) ◽  
pp. 487-493
Author(s):  
M Möllers ◽  
S P Aries ◽  
D Drömann ◽  
B Mascher ◽  
J Braun ◽  
...  

BACKGROUNDPulmonary sarcoidosis is characterised by a mononuclear alveolitis with a predominance of CD4+ T cells and macrophages. We determined the intracellular expression of interferon (IFN)γ, interleukin (IL)-2, tumour necrosis factor (TNF)α, IL-4, IL-5 and IL-10 in CD4+ and CD8+, naive and memory lymphocytes from blood and bronchoalveolar lavage (BAL) fluid using three colour flow cytometry.METHODSEighteen untreated patients with pulmonary sarcoidosis were evaluated and stratified according to whether they had acute or chronic disease.RESULTSSignificantly more T cells expressed Th1 than Th2 type cytokines in both BAL fluid and peripheral blood samples, regardless of clinical presentation. Significantly greater proportions of T cells secreted Th1 type cytokines in BAL fluid than in peripheral blood. Th1 type cytokines were more frequently expressed by peripheral and alveolar T cells in acute disease than in chronic disease. There were no significant differences between CD4+ and CD8+ T cells. Concerning naive and memory lymphocytes, significantly higher CD45RO:CD45RA ratios were found in BAL fluid than in blood, and increased expression of Th2 type cytokines was found in peripheral compared with alveolar memory T cells.CONCLUSIONSOur data support the immunopathogenetic concept of Th1/Th2 imbalance and compartmentalisation in pulmonary sarcoidosis and suggest that the cytokine patterns change during the course of disease. Expression of Th2 type cytokines in memory lymphocytes is decreased in the alveolar compartment compared with peripheral blood.


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