- Mesenteric panniculitis: diagnostic precision. [Journal Article]
- BCBMJ Case Rep 2018 Oct 12; 2018
- A previously healthy 74-year-old woman was admitted with vespertine fever, tremors, shivers and loss of appetite within the previous month. Blood tests revealed an elevated C reactive protein serum l...
A previously healthy 74-year-old woman was admitted with vespertine fever, tremors, shivers and loss of appetite within the previous month. Blood tests revealed an elevated C reactive protein serum level. Serologies for infection were negative. Blood cultures grew no organisms. Colonoscopy revealed normal findings. CT showed typical findings of mesenteric panniculitis with infiltration of mesenteric fat that was circumscribed by hyperattenuating capsule and contained enlarged homogenous lymph nodes. The histopathological analysis from mesenterium revealed non-specific signs of chronic inflammation. On institution of prednisolone, the clinical symptoms subsided, and we replaced it with azathioprine after 1 month. After 12 months of therapy, the patient remained asymptomatic, normalised the serological inflammatory markers and repeat CT revealed normal mesenteric fat.
- Successful azathioprine treatment in an adolescent with chronic enteropathy associated with SLCO2A1 gene: A case report. [Journal Article]
- MMedicine (Baltimore) 2018; 97(41):e12811
- CONCLUSIONS: Physicians should consider CEAS in patients with refractory anemia, hypoproteinemia, and multiple small intestinal ulcers. Why our patient responded to azathioprine but not to corticosteroids is unclear, but azathioprine might benefit some other patients with CEAS.
- Causative Agents of Drug-Induced Pancreatitis: A Nationwide Assessment. [Journal Article]
- PPancreas 2018 Oct 10
- CONCLUSIONS: L-Asparaginase is the most common cause of DIAP in Korea. Serious DIAP is more likely to occur in patients taking multiple medications.
- IgG4-Related Sclerosing Cholangitis Involving the Intrahepatic Bile Ducts Diagnosed with Liver Biopsy. [Journal Article]
- CRCase Rep Pathol 2018; 2018:2309293
- IgG4-related disease is characterized by lymphoplasmacytic inflammation and fibrosis, often leading to mass-forming lesions in different organs. When IgG4-related disease affects the bile ducts, it i...
IgG4-related disease is characterized by lymphoplasmacytic inflammation and fibrosis, often leading to mass-forming lesions in different organs. When IgG4-related disease affects the bile ducts, it is called IgG4-related sclerosing cholangitis. A 74-year-old male complained of dysphagia and abdominal pain. Endoscopic retrograde cholangiography and magnetic resonance cholangiography revealed bile duct changes suspicious of a bile duct carcinoma or cholangitis. Liver biopsy showed storiform fibrosis, lymphoplasmacytic infiltration, obliterative phlebitis, and a portal-based inflammatory nodule with expansion of a portal tract. Hot spots revealed 339 IgG4-positive cells per high power field (HPF) and an IgG4/IgG ratio of 72%. Eight months earlier, an inguinal lymph node had been removed, showing expanded interfollicular zones and increased plasma cells. Hot spots revealed 593 IgG4-positive cells and an IgG4/IgG ratio of 92%. The serum IgG4 of the patient was elevated nearly 10 times upper limit of normal. The diagnosis of IgG4-related sclerosing cholangitis associated with IgG4-related lymphadenopathy was made. There was good response to treatment with prednisolone and azathioprine. The differentiation of IgG4-related sclerosing cholangitis from primary sclerosing cholangitis and bile duct carcinoma is often difficult. Liver biopsy only rarely contributes to this setting, but we describe and report in detail a case where liver biopsy showed a portal-based inflammatory nodule with the characteristic features of this disease.
- Treatment strategies for neuromyelitis optica. [Review]
- CJCi Ji Yi Xue Za Zhi 2018 Oct-Dec; 30(4):204-208
- Neuromyelitis optica (NMO) is an autoimmune demyelinating disease with pathogenic autoantibodies that act against the astrocyte water channel protein, i.e. aquaporin-4: the disease is associated with...
Neuromyelitis optica (NMO) is an autoimmune demyelinating disease with pathogenic autoantibodies that act against the astrocyte water channel protein, i.e. aquaporin-4: the disease is associated with recurrent episodes of optic neuritis (ON) and transverse myelitis, often resulting in severe disability. The main goals in treatment of NMO include acute symptomatic therapy and long-term stabilization of symptoms by preventing relapse. In recent years, ongoing randomized controlled trials in NMO patients have studied evidence for treatment. Briefly, acute-stage management (with pulse therapy using corticosteroids and/or plasmapheresis) and maintenance therapy (including rituximab, mycophenolate mofetil, and azathioprine) have been recommended in some case series and retrospective studies. Because of the high prevalence of liver disease, all NMO patients in Taiwan should be screened for hepatitis B and C before treatment is initiated. Although immunosuppression and plasma exchange are the mainstays of therapy for NMO ON, several selective and potentially therapeutic strategies targeting specific steps in NMO pathogenesis including blockers of NMO-IgG binding and inhibitors of granulocyte function have been evaluated in recent years.
- Intralesional pentoxifylline injection in localized alopecia areata. [Journal Article]
- JCJ Cosmet Dermatol 2018 Oct 09
- CONCLUSIONS: Pentoxifylline intralesional injection is effective, easy to perform with little side effects for the treatment of localized alopecia areata.
- Eosinophilic fasciitis: a case series with an emphasis on therapy and induction of remission. [Journal Article]
- DCDrugs Context 2018; 7:212529
- Eosinophilic fasciitis is an uncommon connective tissue disorder that affects patients of all ages, resulting in significant morbidity. Systemic corticosteroids can induce remission of disease. Howev...
Eosinophilic fasciitis is an uncommon connective tissue disorder that affects patients of all ages, resulting in significant morbidity. Systemic corticosteroids can induce remission of disease. However, there is no universally accepted treatment ladder for eosinophilic fasciitis. This case series evaluates treatment efficacy in patients with eosinophilic fasciitis seen at Wake Forest University Department of Dermatology outpatient clinics. Patient charts were screened using ICD-9 diagnosis code 710.9 (unspecified diffuse connective tissue disease) to identify patients with eosinophilic fasciitis (n=10) seen at our institution. Patients were treated for an average 24 months with a combination of methotrexate and prednisone therapy, unless one or both were contraindicated, with each medication tapered conservatively to prevent disease flares. Alternate treatments included mycophenolate mofetil with prednisone, azathioprine with prednisone, prednisone monotherapy, and methotrexate monotherapy. Disease remission off therapy and on low-dose therapy was 66 and 70%, respectively. Our first-line therapy of concomitant methotrexate and prednisone is well-tolerated and effective for managing patients with eosinophilic fasciitis. Our study was limited to cases seen at a single academic institution.
- Syringomyelia-like syndrome in neuromyelitis optica spectrum disorder complicated with Sjogren's syndrome: a case report. [Journal Article]
- BNBMC Neurol 2018 Oct 09; 18(1):168
- CONCLUSIONS: Although extremely rare, This index patient highlights that syringomyelia could be associated with underlying NMOSD and pSS, and autoimmune disorders should be considered in the initial differential diagnosis, This is very helpful for the therapeutic implications and evaluating curative effect.
- Castleman disease of the hyaline vascular variant transforming to POEMS syndrome as endpoint: a case report. [Journal Article]
- BNBMC Neurol 2018 Oct 09; 18(1):169
- CONCLUSIONS: The diagnosis of POEMS is based on a cluster of disorder involved in varied organs. We report a rare case that triggers the need to consider POEMS syndrome diagnosis for patients carrying Castleman disease and polyneuropathy.
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- Steroid-Responsive Relapsing-Remitting Neutrophilic Encephalitis: A Case Report. [Journal Article]
- JNJ Neuropathol Exp Neurol 2018 Oct 08
- We report a case of a rapidly progressing, relapsing-remitting, steroid-responsive granulocytic encephalitis without any signs of peripheral nervous system or other organ involvement. It apparently h...
We report a case of a rapidly progressing, relapsing-remitting, steroid-responsive granulocytic encephalitis without any signs of peripheral nervous system or other organ involvement. It apparently had an immune-mediated etiology that could not be attributed to any known disease entity. A 22-year-old man presented with rapidly progressive severe neurological symptoms caused by encephalitis. Examination of the cerebrospinal fluid as well as brain biopsy showed extensive accumulation of neutrophilic granulocytes with no hints of an infectious agent. Magnetic resonance imaging revealed multiple T2/FLAIR demarcated lesions. Subsequent to a steroid pulse therapy, the clinical symptoms and imaging abnormalities improved rapidly. Ten months later, the patient experienced a disease relapse, which again responded well to steroids. Forty months after the relapse, he is currently doing well on azathioprine. This case highlights that an immunosuppressive treatment should be considered in patients with extensive neutrophilic encephalitis when no infectious agent is detected. A new immune-mediated relapsing-remitting CNS disease entity might need to be considered.