scholarly journals Multicentric Reticulohistiocytosis Exhibiting Positive HLA-B*07 and HLA-B*08: A Case Report

Medicina ◽  
2020 ◽  
Vol 56 (9) ◽  
pp. 456
Author(s):  
Elena Rezuș ◽  
Maria Alexandra Burlui ◽  
Anca Cardoneanu ◽  
Danisia Haba ◽  
Mihai Danciu ◽  
...  

Multicentric reticulohistiocytosis (MRH) is a rare cause of destructive inflammatory arthritis involving both small, as well as larger joints. We report the case of a 40-year-old Caucasian female with a family history of neoplasia who was referred to our service witha two-month history of inflammatory joint pain. On examination, the patient had inflammatory arthritis, mainly involving the peripheral joints, sacroiliac joint pain, and numerous papulonodular mucocutaneous lesions, including periungual “coral beads”. Imaging tests revealed erosive arthritis with synovitis and tenosynovitis, sacroiliac joint changes, as well as papulonodular mucosal lesions in the nasal vestibule, the oropharyngeal mucosa, and supraglottic larynx. She tested positive for HLA-B*07 (Human Leukocyte Antigen B*07) and HLA-B*08, ANA (antinuclear antibodies), RF (rheumatoid factor), anti-Ro52, anti-SSA/Ro, and anti-SSB/La antibodies. The skin biopsy was suggestive of MRH, showing a histiocyte infiltrate and frequent giant multinucleated cells. The patient exhibited favorable outcomes under Methotrexate, then Leflunomide. However, she displayed worsening clinical symptoms while under Azathioprine. To our knowledge, this is the first case of MRH to exhibit positive HLA-B*07 together with HLA-B*08. The rarity of MRH, its unknown etiology and polymorphic clinical presentation, as well as its potential neoplastic/paraneoplastic, and autoimmune nature demand extensive investigation.

Stroke ◽  
2014 ◽  
Vol 45 (suppl_1) ◽  
Author(s):  
Yasufumi Gon ◽  
Manabu Sakaguchi ◽  
Syuhei Okazaki ◽  
Hideki Mochizuki ◽  
Kazuo Kitagawa

Objective: Previous studies have shown that the prolonged duration of TIA symptoms or ABCD2 score are associated with DWI abnormality, and the presence of DWI abnormality is associated with an increased early risk of stroke. However, there are few reports that show the relation between TIA clinical etiology and DWI abnormality. Our aim of this study is to clarify the prevalence of positive DWI in relation to characteristics of patients and TIA. Methods: The subjects were enrolled from patients who were admitted to our stroke unit within 7 days after symptom onset from January 2006 to July 2013. The diagnosis of TIA was done by NINDS criteria, and we classified TIA etiology by TOAST classification based on clinical symptoms, ECG monitoring, carotid ultrasound, MR angiography and transesophageal echocardiography. All patients underwent DWI-MRI within 7 days after symptom onset. We examined an association between TIA etiology, symptom, duration of symptoms and DWI abnormality. Results: A total of 141 patients (mean 64 years; 63% men) were admitted with TIA during this period. Those included lacuna TIA (n=17, 12.1%), atherothrombotic TIA (n=32, 22.7%), cardioembolic TIA (n=23, 16.3%), TIA due to other causes (n=35, 24.8%), and TIA with unknown etiology (n=34, 24.1%). Prevalence of positive DWI findings were 47.1% in lacunar TIA, 43.7% in atherothrombotic TIA, 52.1% in cardioembloic TIA, 42.8% in TIA due to other causes, and 23.5% in TIA with unknown etiology. DWI abnormality was the most frequent in cardiogenic TIA. In relation to symptom duration, the prevalence of DWI positive findings were 45.2% in less than 1 hour (N=53), 36.6% in 1-3 hour (N=41), 25.0% in 3-6 hour (N=12) and 42.9% in 6-24 hours (N=35). In relation to motor symptoms, there was no difference in prevalence of DWI abnormality between patient with motor symptoms (39.8%, N=113) and without (42.8%, N=28). There was no relation between DWI abnormalities and age, a history of stroke/TIA episode or vascular risk factors. Conclusion: Prevalence of DWI positive findings was high in cardiogenic TIA, and low in TIA with unclassified etiology. There were no relation between DWI abnormality, duration of symptom, and motor symptom.


2017 ◽  
Vol 28 (1) ◽  
pp. 50-52
Author(s):  
Shaymal Sarkar ◽  
Md Daharul Islam ◽  
Marwa Kashem Muna ◽  
SM Tajdit Rahman ◽  
Md Azizul Hoque ◽  
...  

Sarcoidosis is a chronic multisystem disorder of unknown etiology characterized by formation of granulomata within affected organs and consequent distortion of their normal architecture. Typically, these are non-caseating epithelioid granulomata involving organized collections of activated macrophages and T lymphocytes. In countries where tuberculosis is endemic, sarcoidosis is often misdiagnosed and mistreated as tuberculosis. We present case report of a 47-year-old female who presented with 2 years history of recurrent, multiple nodules with occasional joint pain & fever and had received anti-tubercular therapy without any improvement. A diagnosis of sarcoidosis is made finally and she was managed accordingly.Bangladesh J Medicine Jan 2017; 28(1) : 50-52


2010 ◽  
Vol 76 (1) ◽  
pp. 79-84
Author(s):  
Martin Wijkstrom ◽  
Rabih I. Bechara ◽  
Juan M. Sarmiento

Sarcoidosis is a systemic granulomatous disease of unknown etiology affecting patients from all genetic backgrounds. Pancreatic involvement is rare; the first case was described on autopsy in 1937. We present a case of pancreatic sarcoidosis without a history of the disease presenting as biliary obstruction mimicking pancreatic malignancy. We also review the literature with respect to management and outcomes of similar cases. The patient described here presented with all the signs and symptoms of a pancreatic malignancy, which was confirmed on a CT scan; the positron emission tomography scan and the CA 19-9 level were also confirmatory of the suspected diagnosis. In this setting, if the mass looks resectable, a Whipple procedure would be the next logical step. However, such strategy would be aggressive management for a benign condition that could be palliated with diverting rather than resective procedures without changing the outlook of the disease. We suggest keeping a high index of suspicion in patients with a history of the disease if demographic concordance exists.


2014 ◽  
Vol 21 (2) ◽  
pp. 252-254 ◽  
Author(s):  
Koji Shinoda ◽  
Hideaki Asahara ◽  
Taira Uehara ◽  
Katsue Miyoshi ◽  
Satoshi O Suzuki ◽  
...  

We report the first case of an occurrence of multiphasic acute disseminated encephalomyelitis (ADEM) associated with atypical rubella virus infection with no rash and long-term increased titers of serum anti-rubella IgM in a 17-year-old male who had no history of rubella vaccination. He suffered from at least six clinical exacerbations with disseminated hyperintense lesions on FLAIR MR images during the course of 18 months. Repeated methylprednisolone pulse therapy and intravenous immunoglobulin therapy resolved the exacerbations. In patients with multiphasic ADEM of unknown etiology, clinicians should also consider the possibility of preceding infection with rubella virus.


2012 ◽  
Vol 1;15 (1;1) ◽  
pp. E53-E58
Author(s):  
Michael J. DePalma

Background: Recurrent or persistent low back pain (LBP) after surgical discectomy (SD) for intervertebral disc herniation has been well documented. The source of low back pain in these patients has not been examined. Objective: To compare the distribution of the source of chronic LBP between patients with and without a history of SD. Study Design: Retrospective chart review. Setting: Academic spine center. Patients: Charts from 358 consecutive patients were reviewed. Charts noting the absence/ presence of SD in patients who subsequently underwent diagnostic injections to determine the source of chronic LBP were included resulting in 158 unique cases for analysis. Methods: Patients underwent either dual diagnostic facet joint blocks, intra-articular diagnostic sacroiliac joint injections, provocation lumbar discography, or anesthetic injection into putatively painful interspinous ligaments/opposing spinous processes/posterior fusion hardware. If the initial diagnostic procedure was negative, the next most likely structure in the diagnostic algorithm was interrogated. Subsequent diagnostic procedures were not performed after the source of chronic LBP was identified. Outcome: The source of chronic LBP was diagnosed as discogenic pain (DP), facet joint pain (FJP), sacroiliac joint pain (SIJP), or other sources of chronic LBP. Results: Based on a Fisher’s exact test, there was marginal evidence the distribution of the source of chronic LBP differed for those with and without a history of SD (P = 0.080). Posthoc comparisons suggested that patients with a history of SD have a higher probability of DP compared to those without a history of SD (82% versus 41%; P = 0.011). Differences in the probability of FJP, SIJP, or other sources between the SD history groups were not significant. Limitations: Small sample size, restrospective design, and possible false-positive results. Conclusions: This is the first published investigation of the tissue source of chronic LBP after SD. It appears that DP is the most common reason for chronic LBP after SD. If more rigorous study confirms our findings, future biologic treatments may hold value in repairing symptomatic annular fissures after SD. Key words: surgical discectomy, chornic low back pain, discogenic pain, facet joint, sacroiliac joint, low back pain, diagnostic injections, medial branch block, lumbar provcation discography


Author(s):  
Mohammad Reza Mirjalili ◽  
Seyedeh Mahdieh Namayandeh ◽  
Mohammad Hasan Lotfi ◽  
Mohammad Reza Dehghani ◽  
Mohsen Mirzaei ◽  
...  

Introduction: SARS-COV 19 is a new coronavirus that was reported first time in 30 December 2019 in Wuhan, China. The first case of coronary heart disease in Iran was reported with a definitive test on February 18 in Qom province and the first case in Yazd province on 24 February. The World Health Organization (WHO) declared a Covid infection on March 11 a pandemic. The aim of this study was to investigate the seroepidemiology of COVID 19 in Yazd province. Methods: The present study was a cross-sectional population-based study of seroepidemiological type that was conducted to investigate the serological prevalence of Covid-19 in the period of 3 to 20 May 2020 in Yazd Province. Sampling was clustered and it was without age and sex restrictions. Information was collected including demographic information, adherence to health protocols, and history of clinical symptoms of COVID 19."Pishtaz ELISA Kit" was used to evaluate the IgG and IgM antibody titers against Covid 19. Chi-square, analysis of variance and T tests were used via SPSS.version.16 software Results: Mean age of the participants was 34.3± 17.5. Among the study participants, 507 (54.5%) were women. The serological prevalence of definite cases was 14.91% (95% CI: 12.91-16.91%) and suspicious cases were 2.85% (95% CI:1.85-3.85%). Ardakan City with 32% had the highest and Taft, Mehriz, Khatam with 7% had the lowest prevalence of COVID 19 (P <0.05). Conclusion: By June 2020, nearly 18% of the population of the province was definitively or suspiciously infected with COVID 19. Unfortunately, up to 25% of the population does not follow the early prevention protocols of COVID 19.


2005 ◽  
Vol 2 (1) ◽  
pp. 81-84
Author(s):  
Kiyoyuki Yanaka ◽  
Muneyoshi Yasuda ◽  
Kentoaro Furusho ◽  
Hiroyuki Asakawa ◽  
Yuji Matsumaru ◽  
...  

Spontaneous intracerebral hematomas usually produce the sudden onset of devastating neurological symptoms. We describe a case showing slowly progressive clinical symptoms followed by a sudden deterioration caused by two different types of intracerebral hematomas coexisting in the adjacent area. A 72-year-old female with a 2-year history of Alzheimer’s disease and a 3-month history of occasional headache and vomiting was admitted after she experienced the sudden onset of right hemiparesis and a speech disturbance. Neuroradiological examinations demonstrated two different types of intracerebral hematomas coexisting in the left temporal lobe. The patient underwent a left frontotemporal craniotomy. A solid hematoma was found immediately below the cortex and a large hematoma cavity, which contained degraded bloody fluid, was found below the solid hematoma. Histological study demonstrated an arteriovenous malformation (AVM). The AVM may have been responsible for the pathogenesis of these two different types of intracerebral hematomas. Initially, a silent intracerebral hemorrhage from the AVM and liquefaction of the hematoma probably formed the cavity and repeated small subclinical hemorrhages into the cavity during a prolonged period of time may have caused the growth of the inner hematoma. The sudden rupture of the AVM most likely caused the outer solid hematoma, resulting in the abrupt onset of hemiparesis and speech disturbance. This is the first case to demonstrate two different types of intracerebral hematomas coexisting in the adjacent area. A possible mechanism for this rare condition is discussed. Nepal Journal of Neuroscience, Volume 2, Number 1, 2005


2017 ◽  
Vol 14 (3) ◽  
pp. 46-48
Author(s):  
Yam Bahadur Roka ◽  
Mohan Karki

Chronic encapsulated intracerebral hematoma (CE-ICH) is an uncommon pathology that presents with headache, seizure, focal neurological deficits, or as a tumor. Trauma as a cause for CE-ICH is even rare and we believe this is the first case report as “trauma causing chronic encapsulated intracerebral hematoma “search in PubMed did not reveal any results. Repeated micro-hemorrhages in the CM or AVM are supposed to cause this lesion which progress from an earlier encapsulated phase to a thick capsulated stage with edema and clinical symptoms. CT or MRI is the diagnostic modality and it mimics, tumor, AVM, CM, angiomableed, cerebral abscess, metastatic mass or neurocysticercosis. Burr hole, mini-craniotomy, craniotomy, CT guided stereotactic aspiration or endoscopic excision are some options with equally good results. The present case with history of trauma was managed successfully with craniotomy with no recurrence for past one year. Nepal Journal of Neuroscience, Volume 14, Number 3, 2017, page: 46-48


Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Su-Ann Yeoh ◽  
Sharfaraz Salam ◽  
Gerry Christofi ◽  
Vanessa Morris

Abstract Background A 32-year-old Lithuanian lady, with a 6-year history of undifferentiated inflammatory arthritis and autoimmune neutropenia, contacted her rheumatologist reporting a constellation of new symptoms. This began with a month’s history of facial weakness and slurred speech. She progressively experienced nasal regurgitation on drinking fluids, reduced exercise tolerance, as well as difficulty chewing, swallowing, and coughing. Her symptoms initially came on in the evening but subsequently appeared earlier in the day. There were no symptoms suggestive of infection. Her arthritis had been well-controlled on hydroxychloroquine and sulfasalazine. She was previously on methotrexate, but this was discontinued due to plans to conceive. Methods Clinical examination during an urgent outpatient review yielded dysphonic, mildly dysarthric speech. Extraocular movements were normal with no ophthalmoplegia. There was mild lower motor neurone facial weakness and an absent gag reflex. She demonstrated fatiguability on repetitive tongue movements and arm abduction. There were no upper motor neurone or cerebellar signs. Blood tests showed normal inflammatory markers, full blood count, creatine kinase, immunoglobulins, liver, and renal profile. Her anti-nuclear antibody titre, previously weakly positive at time of diagnosis of inflammatory arthritis, was 1:1280 (homogenous). Anti-cyclic citrullinated peptide, rheumatoid factor, and extractable nuclear antigens were negative. Virology screen was unremarkable. Neurology input was sought. Anti-acetylcholine receptor antibody was positive at 5.2nmol/L (normal range 0-0.45nmol/L), anti-muscle specific receptor tyrosine kinase antibody was negative. Nerve conduction studies showed decremental response on repetitive stimulation of her left nasalis, consistent with a post-synaptic neuromuscular junction transmission disorder. There was no evidence of myopathy. Results The clinical symptoms and the investigations led to the diagnosis of myasthenia gravis with predominant bulbar features. Computed tomography showed no evidence of thymoma or thymic hyperplasia. Magnetic resonance imaging of the brain showed no evidence of space-occupying lesion or brainstem abnormality. She initially received pyridostigmine 30mg three times daily with propantheline cover to which she reported a good response. A viral illness led to deterioration of her symptoms of breathlessness and worsening dysphagia resulting in a hospital admission. She received intravenous immunoglobulins 20g daily for three days. Hydroxychloroquine and sulfasalazine were replaced with methotrexate 15mg weekly. She now remains on pyridostigmine 90mg five times daily and 10mg prednisolone daily with good effect. Conclusion Myasthenia gravis is a rare condition (prevalence of 10-20 per 100,000 in the United Kingdom). However, this occurs at a higher prevalence in autoimmune diseases such as rheumatoid arthritis and systemic lupus erythematosus. This case illustrates the importance of remaining vigilant about symptoms which patients may disclose in the rheumatology clinic which may not fit with their known diagnosis. Her previous use of methotrexate for her arthritis could have masked her myasthenic symptoms which became apparent after the drug was stopped for a prolonged period. Disclosures S. Yeoh None. S. Salam None. G. Christofi None. V. Morris None.


2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Palak Arora ◽  
Lorraine Croot

Abstract Case report - Introduction Chikungunya is a tropical arbovirus transmitted by female Aedes Aegypti or Aedes Abopitus mosquitos. It is not indigenous to UK but occurs in epidemics in Africa and Asia. It often presents with pyrexia, arthralgia or arthritis, myalgia and a maculopapular rash and can mimic both peripheral and axial inflammatory arthritis as well as more common forms of viral arthritis. It can also become chronic leading to disabling symptoms. The diagnosis should be considered in all patients presenting with early inflammatory arthritis who have travelled to affected areas. Case report - Case description A 57-year-old female developed sudden onset fever along with a macular rash whilst visiting South East Asia. She then developed widespread joint pains and severe inactivity stiffness, particularly affecting her ankles. The rash and fever settled after a few days, but her arthralgia persisted in her cervical spine and both small and large joints. She had a history of recurrent episcleritis and had been investigated for axial spondyloarthropathy two years previously, but MRI imaging of the spine and sacroiliac joints did not show any inflammatory changes. Examination in the rheumatology clinic confirmed right medial epicondylitis, bilateral shoulder tenderness, tenderness over the extensor tendons of the feet and painful cervical spine movement. Investigations revealed high inflammatory markers; CRP 29 (0-10 mg/L) and ESR 48 (0-15 mm/hr), a positive rheumatoid factor but negative anti CCP antibodies and a normal white cell count. Acute seronegative spondyloarthropathy was suspected but Chikungunya serology was requested at the suggestion of the patient, because of the history of a mosquito bite. IgM and IgG antibodies were positive on immunofluorescence, confirming recent infection. She was initially given intramuscular depomedrone and non-steroidal anti-inflammatory drugs (NSAIDs) with a short response but required oral prednisolone 20mg daily to suppress the inflammation in her feet. An MRI confirmed an ankle effusion and peroneal tenosynovitis. After 6 months her symptoms improved, and she was able to stop prednisolone completely and she remains well 9 months after the initial infection. Case report - Discussion Chikungunya infection causes musculoskeletal symptoms in all affected patients, but the clinical presentation can highly variable, from mild joint pain to erosive arthritis. It can be divided into three phases: incubation phase, acute phase, and chronic phase. The incubation phase varies between one to twelve days after the mosquito bite. The acute phase begins with high fever, headache, polyarthralgia/arthritis, lymphadenopathy, and anorexia. Joint involvement is often distal and symmetrical affecting the hands, wrists, shoulders, knees, ankles, and feet. A maculopapular rash is common. Dengue virus and Zika virus infection can present similarly. Treatment for acute Chikungunya fever is supportive. Analgesic, anti-pyretic and NSAIDs are used for symptom relief. During the chronic phase, infected people develop symmetrical, migratory, oligoarticular or polyarticular arthritis with morning stiffness and joint oedema, which can last from months to years. Our patient had a previous history which was consistent with seronegative spondyloarthropathy, an acute presentation of inflammatory arthritis and results and imaging which supported this diagnosis. The correct diagnosis could easily have been missed if a travel history had not been taken and the patient’s suspicions ignored. The best treatment for chronic Chikungunya arthritis is unclear. NSAIDs are often the first treatment but, as in this case systemic steroids are often necessary. Conventional synthetic DMARDs have also been reported efficacious. Biologic DMARDS have been used in resistant cases. Case report - Key learning points Chikungunya has emerged as a global disease affecting millions of people with significant musculoskeletal morbidity. Any patient has travelled to endemic areas including Africa and Asia, with fever and joint pain should be screened for Chikungunya virus as well as Dengue virus, and Zika virus. Diagnosis is either by RT PCR (positive 0-7 days of infection or Immunoglobulin M (detectable after 5 – 10 day of infection and persists for few months). Treatment is supportive in acute phase, may require low doses of steroids to aid resolution of symptoms. Conventional DMARDS have shown benefit in chronic phase with ongoing synovitis/tenosynovitis. Patients may know more about rare, endemic diseases than their European doctors and their suspicions about potential diagnoses should always be considered.


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