A Case of Eosinophilic Polymyositis Treated with Immunosuppressants

ATYPICAL VARIANT OF GRANULOMATOSIS WITH POLYANGIITIS WITH EOSINOPHILIA

Objective:To explore the correlation between peripheral blood eosinophils and tissue eosinophils in patients with chronic rhinosinusitis with nasal polyps(CRSwNP) in different periods.Method:This trial was a prospective study involved 342 patients with CRSwNP who were treated by endoscopic sinus surgery in the Huai'an First People's Hospital, Nanjing Medical University in 2008, 2012 and 2016. Nasal polyp resection specimens were stained by HE.Tissue eosinophils were counted per high-power field, and peripheral blood eosinophils were collected from blood routine examination before operation.The relationship between peripheral blood eosinophils and tissue eosinophils were evaluated.Result:The correlation between peripheral blood eosinophils and tissue eosinophils was positive in nasal polyps in 2008,2012 and 2016(r= 0.275,0.435,0.505, respectively). AUC values were all greater than 0.7. Conclusion:There is a positive correlation between peripheral blood eosinophils and tissue eosinophils infiltration in patients with CRSwNP. Although the cutoff value is changing, peripheral blood eosinophils can be used as an indicator for predicting eosinophilic CRSwNP.

Not Just Blood: Brain Fluid Systems and Their Relevance to Cerebrovascular Diseases.

BackgroundProtracted febrile myalgia (PFM) is a rare and least well recognized manifestation of familial Mediterranen fever (FMF), characterized by prolonged excruciating muscle pain, tenderness, fever, and elevated levels of acute...

Background:Magnetic resonance imaging (MRI) allows the visualization of both inflammatory and structural lesions of the sacroiliac joints (SIJ) and the spine, playing a pivotal role in the diagnosis and classification...

Background: The accumulation of eosinophils in the lung is a hallmark of asthma. In addition to cytokines such as IL-5 which are essential, chemokines have been implicated in the recruitment of eosinophils to the airway. In particular, eotaxin has been shown to be a selective and potent eosinophil chemoattractant, important in the pathogenesis of allergic disease. The goal of the present study was to define the role of eotaxin-1 in the development of allergen-induced eosinophilic airway inflammation and airway hyperresponsiveness (AHR) to inhaled methacholine (MCh). Methods: Eotaxin-1-deficient mice were sensitized and exposed to a single challenge with allergen. Airway function and airway and tissue as well as peripheral blood and bone marrow eosinophilia were examined 18 and 48 h after the last challenge. Results: Following allergen sensitization and challenge, eotaxin-1-deficient mice developed levels of AHR to inhaled MCh at 18 and 48 h comparable to controls. Further, levels of bronchoalveolar lavage (BAL) and tissue eosinophilia at the same time points were comparable in the two strains of mice. Tissue eosinophilia, assessed by quantitating major basic protein staining cells, preceded BAL eosinophilia in a similar manner. Bone marrow and peripheral blood eosinophilia were unimpaired in deficient mice. Conclusion: The results demonstrate that the major eotaxin, eotaxin-1 is not essential for the development of airway eosinophilia or AHR, implying that other chemokines, alone or in combination, can overcome this deficiency.

Whole-body MR imaging in evaluation of children with juvenile dermatomyositis

A middle-aged male presented with complaints of mild headache and unsteadiness of gait for past 2 years. His physical examination and lab results, including the cerebrospinal fluid (CSF) examination were unremarkable. Magnetic resonance imaging (MRI) of brain was done which revealed a cluster of cysts in the midbrain isointense to CSF on all sequences [Figures 1 and 2]. No enhancement of the cyst walls was seen on post-gadolinium images [Figure 3]. MR spectroscopy did not show any choline, lipid, or amino acid peak in the lesions. These were seen to cause mass effect on aqueduct of Sylvius and mild obstructive hydrocephalus. Review of MRI done before 2 years revealed the same lesions in the mesencephalothalamic region; no change in size or signal intensity was observed in the present scan. The final diagnosis was tumefactive Virchow-Robin spaces causing mild hydrocephalus. The patient was referred to neurosurgery department for further management. A VP shunt was placed with dramatic resolution of symptoms.Figure 1: Axial T1-weighted images show cluster of cysts in the mesencephalic region isointense to cerebrospinal fluid (CSF)Figure 2: Coronal fluid attenuated inversion recovery (FLAIR) image shows CSF isointense cysts in mesencephalothalamic region causing mild hydrocephalus. Periventricular hyperintense signal is due to transependymal seepage and/or microvascular ischemiaFigure 3: Coronal post-gadolinium T1-weighted images do not reveal any enhancement of cysts, consistent with the diagnosis of tumefactive perivascular spacesThe Virchow-Robin or perivascular spaces are pial-lined, fluid-filled spaces accompanying perforating arteries and venules. Typical perivascular spaces occur along lenticulostriate arteries above the anterior perforating substance and anterior commissure. They may occur less commonly in subcortical location, subinsular region, and cerebellum.[1] Rarely they may become enlarged (called tumefactive Virchow-Robin spaces) and cause mass effect and hydrocephalus (especially those found in mesencephalothalamic region); these need to be distinguished from cystic neoplasms.[2] Some authors have proposed the term Virchoma for enlarged mesencephalic perivascular spaces.[3] Tumefactive or cavernous dilation of Virchow-Robin spaces occurring in mesencephalothalamic region may be associated with hydrocephalus. The diagnostic features for tumefactive or cavernous dilation of Virchow-Robin spaces in this case are, an absence of signal from the lesion on the fluid attenuated inversion recovery (FLAIR) imaging, no abnormal signal in the adjacent neuroparenchyma, no contrast enhancement on post-gadolinium scans, and no change in size or appearance over 2 years. The differential diagnosis of tumefactive perivascular spaces includes cystic neoplasms, parasitic cysts, ventricular diverticulae, nonneoplastic neuroepithelial cysts, and mucopolysaccharidosis.[345] Extreme unilateral dilation of Virchow-Robin spaces may also be encountered.[6] Large Virchow-Robin spaces (VRS) have been associated with age, dementia, multiple sclerosis, trauma, hypertension, and incidental white matter lesions.[178] Surgical shunting of the hydrocephalus associated with VR spaces has produced variable clinical results (with improvement in gait disturbances, bradykinesia, and urinary symptoms). We conclude that mesencephalic tumefactive Virchow Robin spaces can cause obstructive hydrocephalus, and rare manifestations of a common entity should always be considered.

To describe the magnetic resonance (MR) imaging findings in diabetic patients with muscle infarction and to describe commonly associated clinical features. The MR imaging studies of 21 patients with diabetic muscle infarction were reviewed retrospectively. Of the 21 patients, 12 were women, and nine were men; the mean age was 48 years (range, 30-77 years). Eight patients had bilateral lower-extremity involvement; six had involvement confined to the right lower extremity and seven to the left. The thigh was involved in 17 patients (81%). One or more of the musculi vastus, the most frequently affected muscle group, were affected in 16 patients (76%). Four patients (19%) had isolated calf involvement. MR imaging studies showed diffuse enlargement of involved muscle groups and partial loss of normal fatty intermuscular septa. MR imaging also allowed identification of areas of subfascial fluid in 16 patients (76%) and subcutaneous edema in 19 patients (90%). MR imaging showed involved muscle groups best with T2-weighted, inversion-recovery, and gadolinium-enhanced sequences, where the infarcted muscles appeared diffusely hyperintense compared with adjacent muscles. Comparison of T2-weighted and gadolinium-enhanced MR images of nine patients showed enlarged, enhancing muscles in all patients and small, focal, rim-enhancing fluid collections in six of nine patients (66%). Diabetic muscle infarction is suggested in diabetic patients with sudden onset of severe pain in the thigh or calf muscles who have MR imaging findings of diffuse edema and swelling of multiple thigh and calf muscles (often in more than one compartment).

Objective To analyze the distribution of Virchow-Robin spaces (VRS) in migraine by MRI, and to study the effects of the duration of the disease, the attack frequency and the migraine with or without aura on the number of VRS in order to provide imaging support for migraine diagnosis. Methods Fifty migraine patients were enrolled as migraine group and 50 healthy people as control group during January 2013 to December 2016 from Department of Neurology, the Second Affiliated Hospital of Zhengzhou University. The number of VRS in the fronto-parietal subcortical white matter, semioval central, and basal ganglia areas was calculated and compared between groups and within the group by performing a MRI scan of the same sequence, and the impact of the history of migraine, the attack frequency and the migraine with or without aura on the number of VRS was investigated. Results The VRS were found in 48 cases in the migraine group, accounting for 96%, significantly higher than in the control group (41 cases, accounting for 82%), the difference being statistically significant (χ2=5.00, P<0.05). In the migraine group, the sum of the number of VRS (13.00(6.75, 20.00)) was significantly higher than that of the control group (8.00(5.00, 12.00); Z=3.33, P<0.01). In the migraine group the VRS numbers in the fronto-parietal subcortical white matter, semioval central and basal ganglia areas were 6.00(4.00, 12.00), 2.00(0.00, 4.00)and 4.00 (2.00, 6.00) respectively, while the numbers of VRS in the same areas of the control group were 0.00(0.00, 2.00), 2.00(0.75, 4.00)and 4.00(3.50, 6.00). The total number of VRS in different areas was significantly different within the two groups (migraine group χ2=39.86, P<0.01; control group χ2=40.15, P<0.01). In the migraine group, the VRS was mainly located in fronto-parietal subcortical white matter, whereas in the control group the VRS was mainly distributed in the basal ganglia. The total number of VRS in the migraine with aura group (20.00 (14.50, 26.00)) was more than that in the migraine without aura group (11.00 (6.00, 20.00); Z=2.52, P=0.02). The numbers of VRS in the fronto-parietal subcortical white matter, semioval central and basal ganglia areas of the migraine with aura group were 12.00(9.00, 14.00), 2.00(2.00, 6.00) and 4.00(2.50, 7.50) respectively; The numbers of VRS in the same areas of the migraine without aura group were 6.00(4.00, 10.00), 1.00(0.00, 4.00) and 4.00(2.00, 6.00)respectively; The numbers of VRS in different areas within the two groups were significantly different (with aura group χ2=16.31, P<0.01; without aura group χ2=29.48, P<0.01). There were statistically significant differences in the number of VRS among migraine without aura patients with different duration and frequency of episodes. Conclusions The incidence rate of perivascular space in migraine is high. VRS is mainly distributed in the fronto-parietal subcortical white matter, which may provide an imaging assistant basis for the diagnosis of migraine. Migraine with aura is more prone to VRS than those without aura. The disease course and the attack frequency have a certain impact on occurrence of VRS. Key words: Migraine disorders; Migraine with aura; Migraine without aura; Magnetic resonance imaging; Perivascular space

BackgroundMRI is the most widely used imaging procedure to assess disease activity of the idiopathic inflammatory Myopathies (IIM). Thigh muscle oedema, detected by T2-weighted and fat-suppressed (short tau inversion recovery,...

The purpose of this study was to compare the macroscopic and microscopic findings of experimentally induced inflammatory lesions in jejunum and ileum with magnetic resonance imaging (MRI) findings. Inflammatory small bowel lesions were experimentally induced in six pigs. Bowel segments in jejunum and ileum were isolated, and a solution with trinitrobenzenesulfonic acid and ethanol (TNBS-EtOH) was installed. MRI of the small bowel was performed 7 days after surgery. Before the MRI examination, a 6% mannitol solution was installed through a nasogastric tube. The MRI protocol consisted of single-shot turbo spin echo T2 sequences, steady state free precession (BFFE) sequences, and a 3D T1 gradient echo sequence with fat saturation and intravenous contrast. The following image findings were evaluated: increased bowel wall thickness (BWT), increased bowel wall enhancement (BWE), and bowel stenosis. After the MRI examination, the animals were sacrificed. The small bowel was removed and examined macroscopically and microscopically. Inflammatory lesions developed in jejunum and ileum in all animals. The lesions were visible macroscopically and microscopically. The microscopic findings consisted of variable degrees of inflammation, ulcer formation, and fibrosis. In jejunum the inflammatory lesions were not diagnosed with MRI, except in one pig with a bowel necrosis probably caused by an intramural injection or leakage of the TNBS-EtOH solution. In ileum the bowel wall thickness was increased and the inflammatory lesions were diagnosed with MRI. In conclusion, the inflammatory lesions were visible macroscopically and microscopically. Lesions in ileum had increased BWT and were possible to image with MRI. Lesions in jejunum had normal BWT and were not diagnosed with MRI, except in one pig with increased BWT probably caused by complications to the installation of TNBS-EtOH.

The 2019 Society for Hematopathology and European Association for Haematopathology Workshop reviewed the spectrum of neoplastic, nonneoplastic, and borderline entities associated with reactive eosinophilia in tissue. The workshop panel reviewed 46 cases covered in 2 workshop sessions. The 46 cases were presented with their consensus diagnoses during the workshop. Reactive eosinophilia in lymph nodes and other tissues may be accompanied by or be distinct from peripheral blood eosinophilia. Reactive etiologies included inflammatory disorders such as Kimura disease and IgG4-related disease, which may show overlapping pathologic features and reactions to infectious agents and hypersensitivity (covered in a separate review). Hodgkin, T-cell, and B-cell lymphomas and histiocytic neoplasms can result in reactive eosinophilia. The spectrum of these diseases is discussed and illustrated through representative cases. Reactive eosinophilia in lymph nodes and tissues may be related to both nonneoplastic and neoplastic lymphoid proliferations and histiocytic and nonhematolymphoid processes. Understanding the differential diagnosis of reactive eosinophilia and the potential for overlapping clinical and pathologic findings is critical in reaching the correct diagnosis so that patients can be treated appropriately.

Objective. To develop the optimal mode of spine evaluation with magnetic resonance image (MRl) in pts with ankylosing spondylitis (AS) and to study relationship between MR! signs of spinal inflammatory lesions (IL), spondylitis duration and clinical features of AS activity. Material and methods. MRl was performed in 36 pts (22 male, 14 female) fulfilling the modified NY criteria of AS. Median age of pis was 26 years (range 19 - 55), Median AS duration - 8 years (range 1,8 - 24). 34 (97%) pts were HLA-B27 positive. 21 (64%) pts had high AS activity - median BASDAI 40 (range 10 - 77). 92% of pts had inflammatory spine pain (VAS>20 mm) and 61% of pts had night pain. Median inflammatory pain duration had been defined separately for every part of the spine assessed by MRl. Median duration of axial pain was 36 months (range: 1-240). MR-scanning (Magnetom Symphony, Siemens, 1.5 T) was performed inTl, T2 and T2-FS (fat signal suppression) modes. IL scoring was done only in 29 pts evaluated in both sagittal and axial planes. We used two scoring methods: 1) individual IL score of the each spine element (vertebral bodies, processes, arches, zygapophyseai, costovertebral and costotransverse joints, ligaments), and 2) separate IL scoring in the vertebral bodies and posterior spinal elements in order yes/no”. Results. 50 MRl images of different parts of the spine (8 cervical, 30 thoracic and 12 lumbar) have been obtained in 36 pts. Spine IL were found in 35 pts. 26% of all IL were revealed in axial planes. 3 pts with short AS duration had IL only on axial slices (zygapophyseai lumbar joints, costotransverse joints, processes). IL were revealed more often in thoracic (average score: 7.1), than in lumbar (3.7) and cervical (2.1) spine. In most (26 from 29 pts, 90%) pts IL were found in painful parts of spine. There was no IL score difference between pts(n=12) with low (BASDAI 40; n=17) AS activity. Me and range were 4 (1.8-10.3) and 6 (4-16), respectively; p=0.35. There was also no difference in percent of images with IL between pts with short (Me: 4 months, range: 1-18; n= 10) and prolonged (Me: 54 months, range: 24-180; n=16) duration of spondylitis (100% and 94% of images, respectively). However, pts with early spondylitis had significantly more IL in posterior spinal structures than in vertebral bodies (92.3% and 23.1% images, respectively; p<0,001). Conclusion. Inflammatory MRl lesions are frequently observed in pts with active AS, more often in thoracic spine, and independently of spondylitis duration. Inflammatory MRl lesions in early spondylitis are revealed more often in posterior structures of spine. These results show the necessity to obtain MRl scans for early diagnosis of AS not only in sagittal but also in axial plane.

Eosinophilic myositis is characterised by peripheral blood eosinophilia and eosinophilic infiltration in muscles, and is comprised three subtypes: focal eosinophilic myositis (FEM), eosinophilic polymyositis, and eosinophilic perimyositis. Muscle involvement of FEM is usually limited to the lower legs, and pulmonary complications have not been reported. We report a rare case of FEM in the left adductor pollicis complicated by lung lesions. A 37-year-old woman developed swelling of the first web space in the left hand. Magnetic resonance imaging (MRI) of the left hand demonstrated increased signal on fat-suppressed T2-weighted imaging in the left adductor pollicis. A muscle biopsy specimen demonstrated perimysial and endomysial infiltration of mononuclear cells and eosinophils, and necrosis and regeneration of muscle fibres. Chest computed tomography (CT) revealed upper-lobe–dominant ground-glass opacities (GGO). Both focal myositis and pulmonary lesions improved without treatment. This case suggests that FEM could involve adductor pollicis and have pulmonary lesions. In this case, myositis and GGO resolved spontaneously. Some FEM cases treated with glucocorticoids were reported in the past. Further studies are required to determine whether patients with FEM require therapeutic intervention.