Fatal Kikuchi–Fujimoto disease associated with SLE and hemophagocytic syndrome: a case report

Kikuchi-Fujimoto disease (KFD) or histiocytic necrotizing lymphadenitis is a rare, benign, autoimmune condition characterized by lymphadenopathy, fever, and neutropenia. KFD can frequently mimic various diseases including infection, malignancy, and autoimmune disease. KFD has also been infrequently reported in association with SLE, which can be diagnosed previously, simultaneously, or after the diagnosis of SLE. The author presents here the first case of fatal KFD simultaneously occurred with SLE complicated with hemophagocytic syndrome and severe infection. The recognition of this association is of significance for management of affected patients with early intensive immunosuppressive therapy for favorable outcome.     

Ankylosing spondylitis coexists with rheumatoid arthritis and Sjögren’s syndrome: a case report with literature review

Clinical Rheumatology - Rheumatoid arthritis (RA), ankylosing spondylitis (AS), and Sjögren’s syndrome (SS) are the most common rheumatic diseases. However, a coexistence of AS and RA or...     

Ankylosing spondylitis occurring in a patient with Behçet disease after chronic brucellosis infection: A case report and review of the literature

Behçet disease and ankylosing spondylitis are rarely seen together. This report presents a rare case of ankylosing spondylitis occurring in a patient with Behçet disease after chronic brucellosis infection. Infectious agents have long been considered one of the triggers for autoimmune and autoinflammatory diseases. Therefore, rheumatic processes should be kept in mind, especially in treatment-resistant cases.     

Comment on: Ankylosing spondylitis coexists with rheumatoid arthritis and Sjögren’s syndrome: a case report with literature review

Clinical Rheumatology -     

Kikuchi–Fujimoto disease developed into autoimmune disease: a report of two cases

We report herein the pathological findings and clinical courses of two cases of Kikuchi–Fujimoto disease (KFD) that developed into autoimmune diseases. The patients are currently undergoing treatment for a disease similar to Sjogren’s syndrome and systemic lupus erythematosus/mixed connective tissue disease. KFD is not an independent condition and most likely develops due to an autoimmune mechanism. Pediatricians should pay careful attention to KFD and encourage long-term follow-up in patients with this condition.     

Enthesitis-related arthritis in Kikuchi–Fujimoto disease

Histiocytic necrotizing lymphadenitis or Kikuchi–Fujimoto disease (KFD) is a rare, benign and self-limiting disorder that characteristically presents with fever and cervical lymphadenopathy. Articular manifestations in the form of arthralgias are common but frank arthritis is distinctly rare and dactylitis has not been reported yet. Herein, we describe a young boy who presented with arthritis and dactylitis as the initial manifestation of KFD. A 14-year-old boy presented with a two-week history of fever, generalized lymphadenopathy and asymmetric polyarthritis, enthesitis and dactylitis of the toes. Two years earlier he presented with arthritis of the knee and ankle joints, which lasted for 12 months. However, he had been asymptomatic for one year. Investigations revealed anemia, leukopenia and raised acute phase reactants. Work-up for infectious etiology, systemic lupus erythematosus and leukemia and lymphoma was negative. Excision biopsy of the cervical lymph node confirmed KFD. Fever, lymphadenopathy and leukopenia dissipated with nonsteroidal anti inflammatory drug therapy, but the arthritis persisted. A trial of methotrexate led to the resolution of the arthritis.     

Diabetes mellitus with hepatic infarction： A case report with literature review

Hepatic infarction rarely occurs due to the double supply of arterial and portal inflow. A 53-year-old man with diabetes mellitus developed multiple hepatic infarctions after an episode of fever and diarrhea. The infarction was documented by pathology after partial liver resection. Several causes of hepatic infarction may present in this patient: dehydration and hypotension caused by fever and diarrhea, type 2 diabetes and administration of glibenclamide, diabetic ketoacidosis and widespread atherosclerosis. We suggest that diabetic patient with elevated liver enzyme should be considered the possibility of hepatic infarction.     

Presacral ganglioneuroma: A case report and review of literature

Presacral ganglioneuromas are so rare benign tumors that only 17 cases have been reported in the literature. They are abdominal masses growing slowly and differential diagnoses have to be considered. Surgical resection is important for definitive diagnosis because it represents the only therapeutic choice. Because of the benign nature of ganglioneuroma, adjuvant chemo-or radiotherapy is not indicated but regular follow-up is necessary for an early diagnosis of potential local recurrence. We report a case of a 64-year-old man with a presacral ganglioneuroma.     

Rectal carcmosarcoma: A case report and review of literature

A 60-years old male was admitted to our department for investigation of constipation and hypogastric discomfort intensified during defecation of a few weeks duration. The cause proved to be a rectal carcinosarcoma that was treated by abdominoperineal resection and postoperative chemo-radiotherapy. The patient died 6 months later due to hepatic failure, showing evidence of disseminated disease. In general colonic carcinosarcomas constitute a rare category of malignant neoplasms whose nature is still incompletely understood. No specific treatment guidelines exist. Surgery is the mainstay of treatment and regardless of the addition of adjuvant therapy the prognosis is very poor. Systematic genetic analysis may be the clue for understanding the pathogenesis of these mysterious tumors.     

A case report of systemic lupus erythematosus combined with Castleman’s disease and literature review

Although lymph node enlargement is common in active systemic lupus erythematosus (SLE), lymph node examination is frequently ignored in the diagnosis of SLE. Clinical presentation and abnormal laboratory findings are often sufficient for SLE diagnosis, not to mention that the specific histological finding of lymph node necrosis in SLE is rarely seen, and the follicular hyperplasia is usually considered as nonspecific. However, since the late 1990s, a few cases of SLE lymphadenopathy have been reported exhibiting a Castleman’s disease (CD) morphology, which was discovered in lymph node biopsies. Here we report a similar case of SLE combined with CD in a 23-year-old girl who displayed systemic symptoms, including systemic lymphadenopathy and abnormal laboratory findings indicating the active phase of SLE. A biopsy of neck lymphnodes showed histopathological features of CD. The patient responded very well to the prednisolone treatment. Based on the related literature review, we would like to stress the possibility of CD in patients with SLE lymphadenopathy.     

Gitelman syndrome with Graves’ disease leading to rhabdomyolysis: a case report and literature review

Minimal change disease (MCD) is a major cause of nephrotic syndrome (NS) in children and a minority of adults. The higher tendency to relapse put patients at risk for prolonged exposure to steroids and other immunosuppressive agents. B cell depletion with rituximab (RTX) may be beneficial to the treatment and prevention of frequently relapsing MCD. Therefore, this study aimed to verify the therapeutic/preventive effects of low-dose RTX on the relapse in adult with MCD. A total of 33 adult patients were selected for the study, including 22 patients with relapsing MCD in relapse treatment group who were treated with low-dose RTX (200 mg per week × 4 following by 200 mg every 6 months) and 11 patients in relapse prevention group with complete remission (CR) after steroid therapy were treated with RTX (200 mg ×1 every 6 months) for preventing the relapse of MCD. Of the 22 patients with MCD in relapse treatment group, there were 21 cases (95.45%) of remission [2 (9.09%) partial remission (PR), 19 (86.36%) CR], 1 (4.56%) no remission (NR) and 20 (90.90%) relapse-free. The Median duration of sustained remission was 16.3 months (3, 23.5 months, inter quartile range (IQR)). 11 patients in the relapse prevention group during a follow-up of 12 months (9–31 months) had no relapse. The average dose of prednisone in two groups after RTX treatment was significantly lower than before treatment. The results of this study suggested low-dose RTX can significantly reduce relapse rate and steroid dose in adults with MCD with fewer side effects. Low-dose RTX regimens may be beneficial for the treatment of relapsing MCD in adults and may be the preferred regimen for patients at high risk for the development of adverse events from corticosteroids.     

Addison’s disease with pituitary hyperplasia: a case report and review of the literature

This case study describes a 33-year-old man with Addison’s disease who presented with increased plasma adrenocorticotropic hormone (ACTH), normal plasma cortisol, and absent diurnal rhythms. Magnetic resonance imaging (MRI) indicated pituitary hyperplasia. Conventional hydrocortisone replacement therapy may not inhibit high ACTH levels in the morning; however, replacing hydrocortisone with dexamethasone achieved good therapeutic results.     

Late onset fulminant Wilson’s disease: A case report and review of the literature

Wilson’s disease (WD) is an autosomal recessive inherited disorder of hepatic copper metabolism. WD can be present in different clinical conditions, with the most common ones being liver disease and neuropsychiatric disturbances. Most cases present symptoms at < 40 years of age. However, few reports exist in the literature on patients in whom the disease presented beyond this age. In this report, we present a case of late onset fulminant WD in a 58-year-old patient in whom the diagnosis was established clinically, by genetic analysis of the ATP7B gene disclosing rare mutations (G1099S and c.1707+3insT) as well as by high hepatic copper content. We also reviewed the relevant literature. The diagnosis of WD with late onset presentation is easily overlooked. The diagnostic features and the genetic background in patients with late onset WD are not different from those in patients with early onset WD, except for the age. Effective treatments for this disorder that can be fatal are available and will prevent or reverse many manifestations if the disease is discovered early.     

Liver cell adenoma： A case report with clonal analysis and literature review

We report a case of liver cell adenoma (LCA) in a 33-year-old female patient with special respect to its clonality status, pathogenic factors and differential diagnosis. The case was examined by histopathology, immunohistochemistry and a clonality assay based on X-chromosomal inactivation mosaicism in female somatic tissues and polymorphism at androgen receptor focus. The clinicopathological features of the reported cases from China and other countries were compared. The lesion was spherical, sizing 2 cm in its maximal dimension. Histologically, it was composed of cells arranged in cords, most of which were two-cell-thick and separated by sinusoids. Focal fatty change and excessive glycogen storage were observed. The tumor cells were round or polygonal in shape, resembling the surrounding parenchymal cells. Mitosis was not found. No portal tract, central vein or ductule was found within the lesion. The tumor tissue showed a positive reaction for cytokeratin (CK) 18, but not for CK19, vimentin, estrogen and progesterone receptors. Monoclonality was demonstrated for the lesion, confirming the diagnosis of an LCA. Clonality analysis is helpful for its distinction from focal nodular hyperplasia.     

Brunner's gland adenoma of duodenum:A case report and literature review

AIM:To analyze the clinicopathological features of Brunner'sgland adenoma of the duodenum.METHODS:A rare case of Brunner's gland adenoma of theduodenum was described and related literature was reviewed.RESULTS:Brunner's gland adenoma of the duodenumappeared to be nodular hyperplasia of the normal Brunner'sgland with an unusual admixture of normal tissues,includingducts,adipose tissue and lymphoid tissue.We suggestedthat it might be designated as a duodenal hamartoma ratherthan a true neoplasm.CONCLUSION:The most common location of the lesion isthe posterior wall of the duodenum near the junction of itsfirst and second portions.It can result in gastrointestinalhemorrhage and duodenal obstruction.Endoscopic polypectomyis a worthy treatment for benign Brunner's gland adenomas,as malignant changes in these tumors have never been proven.     

Radiological diagnosis of duodenocaval fistula:A case report and literature review

Duodenocaval fistula(DCF) is an uncommon but lethal clinical entity.The high mortality has been attributed to the difficulty of diagnosis before attempts at definitive therapy.In this case report,we describe a patient with a series of computed tomography(CT) examinations over a 2-mo period in hospital.A low-density air bubble appeared in the inferior vena cava(IVC) on the second day in hospital and became clear on day 19,and gradually enlarged.Magnetic resonance imaging(MRI) also clearly demonstrated a high...     

Presacral ganglioneuroma： A case report and review of literature

Presacral ganglioneuromas are so rare benign tumors that only 17 cases have been reported in the literature. They are abdominal masses growing slowly and differential diagnoses have to be considered. Surgical resection is important for definitive diagnosis because it represents the only therapeutic choice. Because of the benign nature of ganglioneuroma, adjuvant chemoor radiotherapy is not indicated but regular follow-up is necessary for an early diagnosis of potential local recurrence. We report a case of a 64-year-old man with a presacral ganglioneuroma.     

Rectal carcinosarcoma： A case report and review of literature

A 60-years old male was admitted to our department for investigation of constipation and hypogastric discomfort intensified during defecation of a few weeks duration. The cause proved to be a rectal carcinosarcoma that was treated by abdominoperineal resection and postoperative chemo-radiotherapy. The patient died 6 months later due to hepatic failure, showing evidence of disseminated disease. In general colonic carcinosarcomas constitute a rare category of malignant neoplasms whose nature is still incompletely understood. No specific treatment guidelines exist. Surgery is the mainstay of treatment and regardless of the addition of adjuvant therapy the prognosis is very poor. Systematic genetic analysis may be the clue for understanding the pathogenesis of these mysterious tumors.     

Kikuchi-Fujimoto’s disease associated with systemic lupus erythematosus: case report and review of the literature

Kikuchi-Fujimotos disease (KFD) or histiocytic necrotising lymphadenitis is a benign and self-limited disease, of unknown aetiology, which affects mainly young women. It presents with localised lymphadenopathy, predominantly in the cervical region, accompanied by fever and leukopenia in up to 50% of the cases. KFD has been rarely described in association with systemic lupus erythematosus (SLE), and its diagnosis can precede, postdate or coincide with the diagnosis of SLE. We present a patient with the diagnosis of SLE characterised by arthritis, leukopenia, malar rash, photosensitivity and positive ANA, besides cervical lymphadenopathy whose biopsy was compatible with KFD, which improved after using prednisone. Although the presence of lymphadenopathy is not uncommon in SLE patients, particularly in the phases of disease activity, the concomitance with KFD has rarely been reported in the literature. Its recognition is necessary because one can avoid laborious investigation for infectious and lymphoproliferative diseases.     

Pituicytoma in a patient with Cushing’s disease: case report and review of the literature

There are scant prospective studies defining improvements in critical outcome measures with hormone replacement in hypopituitarism secondary to brain injury. We review the tests of cognition and physical function and summarize their use for subjects that are deficient in anterior hormone production during anterior pituitary hormone replacement in brain injury and propose these as the minimal tests that are feasible for a physician to perform in a clinical setting. We summarize the studies conducted to assess outcome measures after brain injury and also report preliminary findings for improvements in cognition and physical function in subjects with brain injury and GH deficiency.