Localised Hyaline Vascular Type of Castleman’s Disease Mimicking Adult-Onset Still’s Disease

A previously healthy 18-year-old boy presented with daily spiking fever, polyarthritis, and evanescent skin rashes, as well as hepatomegaly and Raynaud’s phenomena for 2 months. He was initially diagnosed with adult-onset Still’s disease (AOSD). During the period of follow-up, intermittent fever and migratory polyarthritis persisted and an insidiously growing mass over the right axillary region was noted 1 year after the diagnosis of AOSD. Excisional biopsy of the mass revealed a group of lymph nodes with histological features of the hyaline vascular type of Castleman’s disease. The patient’s symptoms disappeared soon after excision of the lymph nodes. Received: 4 January 1999 / Accepted: 4 June 1999     

Small Bowel Lesions Mimicking Crohn’s Disease

Purpose of Review

Not all injuries of the terminal ileum are Crohn’s disease. It is the purpose of this review to consider the differential diagnosis of other acute and chronic ileal lesions.

Recent Findings

The recognition of a granulomatous disease of the terminal ileum, distinct from tuberculosis, dates back over 85 years and perhaps much farther, but over the past decades, many other clinical pathologic entities have been described that are neither tuberculosis nor Crohn’s eponymous regional enteritis. In recent years, the catalog of lesions mimicking Crohn’s disease of the small bowel and proposals for differential diagnosis and treatment have expanded to include newly reported appendiceal pathology, primary cancers and lymphomas of the intestine, unexpected metastases from distant organs, unusual infections, vasculitides and other ischemic conditions, Behçet’s disease, endometriosis, and drug reactions.

Summary

A diagnosis of Crohn’s disease should not be a reflex action in the face of small bowel structural or inflammatory lesions without consideration of pathology in adjacent organs, primary and metastatic lesions of the small intestine, infections, vascular diseases, infiltrative diseases, drug injury, or other “idiopathic” conditions.

     

Tuberculous Spondylitis (Pott’s Disease)

A 46-year old Caucasian male nurse presented with a 12-month history of myalgias and leg weakness, remitting thoracic and lumbar back pain, fever, weight loss and night sweats. Blood tests revealed elevated inflammatory activity and anemia. Suspecting vasculitis, an 18F-fluorodeoxyglucose positron-emission tomographic (18FDG-PET) scan was performed, which demonstrated enhanced uptake in the thoracic and lumbar spine (Figure a). Consistent with these findings, computed tomography (CT) of the spine revealed a large paravertebral abscess (Figure b and Figure c, white arrow) as well as severe spondylodiscitis with destruction of the vertebrae Th 7 and L 1 (Figure c, black arrow). Additionally, CT detected osteolytic lesions of the pelvis, multiple pulmonary lesions and mediastinal lymphadenopathy. The paravertebral abscess was drained and histological examination (Figure d, HE, original magnification x 100) demonstrated epitheloidgranulomatous (black arrow) and necrotizing inflammation (white arrow). Polymerase chain reaction and culture of the paravertebral and sputum specimens revealed infection with Mycobacterium tuberculosis. Radiological and microbiological findings thus indicated the diagnosis of tuberculous spondylitis (Pott's disease) resulting from disseminated postprimary pulmonary tuberculosis. Treatment was started with isoniazid, ethambutol, rifampicin and pyrazinamide. Symptoms improved markedly after several weeks and the patient recovered well. CT scans performed after 3, 6 and 9 months demonstrated regression of all lesions.The spine is the most frequent site of osseous tuberculous with predominant affection of the upper lumbar and lower thoracic spine. The infection commonly begins in the anterior part of the vertrebal bodies and subsequently involves the disk space, adjoining ligaments and soft tissues. Spinal tuberculosis is frequently accompanied by paraspinal abscesses.     

Thromboangiitis Obliterans (Buerger’s Disease)

Thromboangiitis obliterans (TAO) is a devastating nonatherosclerotic disease that often leads to digit and limb loss as well as intractable ischemic rest pain. Patients with TAO are uniformly heavy users of tobacco. This disorder is characterized as a miscellaneous form of vasculitis affecting small- and medium-sized arteries and veins. TAO causes painful ischemic ulcers of the digits and unusually painful and inflammatory superficial thrombophlebitis. The key to early diagnosis of TAO is a high clinical index of suspicion in the appropriate patient scenario, exclusion of any other potential cause, abnormal results from an Allen's test, and arteriographic findings of segmental digital arterial occlusions with "corkscrew" collateral vessels. The primary and clearly most effective therapy for TAO is cessation of the use of all tobacco products, with avoidance of any environmental tobacco smoke inhalation. There has been recent enthusiasm for prostaglandin E therapy and the intramuscular injection of angiogenic growth factors. For patients in whom the disease is identified late or for those who are unable to discontinue cigarette smoking, however, a frequent result is multiple limb amputations.     

Ganglioneuromatosis: An Unusual Cause of Ileal Stricture Mimicking Crohn’s Disease

Ileal idiopathic forms of ganglioneuromatosis in adults are extremely rare and represent a challenging pathologic condition for the clinician. We present two cases of ileal ganglioneuromatosis consisting of stricturing lesions that mimicked clinical and radiologic features commonly observed in Crohn’s disease patients with ileal involvement.     

Atypical Presentation of Hashimoto’s Disease in an Adolescent: Thyroid-Associated Ophthalmopathy

Hashitoxicosis is generally differentiated from Graves’ hyperthyroidism by its shorter course and absence of ophthalmopathy. In this case report, we describe an adolescent girl who presented with significant clinical findings of hyperthyroidism, a diffuse goiter with homogenously increased uptake in scintigraphy, and with ocular findings of ophthalmopathy. The thyroid stimulating hormone receptor antibody test was positive, and the family history revealed thyroid-associated ophthalmopathy. Clinical findings supported the diagnosis of Hashimoto’s disease (HD) in the follow-up period. Radioactive iodine uptake investigation was found to be a reliable method for differential diagnosis. Attention was drawn to the rarity of pediatric cases of HD who present with ophthalmopathy.     

Concurrent Hodgkin’s Disease (Mixed Cellularity Type) and T-Cell Chronic Lymphocytic Leukemia/Prolymphocytic Leukemia

We describe a patient with leukopenic T-cell chronic lymphocytic leukemia/prolymphocytic leukemia (T-CLL/PLL), according to the Revised European-American Classification of Lymphoid Neoplasms. This patient simultaneously developed classic Hodgkin's disease (HD), a combination previously unreported. The leukemic cells were small and mature, did not have cytoplasmic granulation, and appeared similar to B-cell chronic lymphocytic leukemia. Immunophenotyping of the bone marrow-infiltrating cells revealed a postthymic suppressor/cytotoxic phenotype of CD2+, CD3+, CD4, CD5+, CD8+, CD25-, TCR-alpha beta. A lymph node biopsy showed the histological features of HD (mixed cellularity) with infiltrating CD8+ lymphocytes, and immunohistochemical examination revealed the following phenotype of Reed-Sternberg cells: LeuM1/CD15+, BerH2/CD30+, L26/PanB-, UCHL-1/CD45RO-, cyCD3-, CD4, CD8-, CD20-, CD79a-, EMA-, EBER-1+, LMP-1+. Southern blot analysis of the bone marrow and lymph node revealed the same rearrangement of bands of T-cell-receptor genes. Although the HD was treated with chemotherapy that resulted in complete remission, the T-PLL/CLL took an indolent course. This case may suggest the existence of a subtype of T-CLL/PLL with leukopenia and an indolent clinical course. Both diseases were believed to be independent and not a transformation of one to the other.     

Can hyperuricemia predict glycogen storage disease (McArdle’s disease) in rheumatology practice? (Myogenic hyperuricemia)

Clinical Rheumatology - Gout disease is an inflammatory arthritis that arises due to the accumulation of monosodium urate crystals (MSU) around the joints and in tissues. Clinical manifestation of...     

Dysphagia in Parkinson’s Disease

More than 80 % of patients with Parkinson’s disease (PD) develop dysphagia during the course of their disease. Swallowing impairment reduces quality of life, complicates medication intake and leads to malnutrition and aspiration pneumonia, which is a major cause of death in PD. Although the underlying pathophysiology is poorly understood, it has been shown that dopaminergic and non-dopaminergic mechanisms are involved in the development of dysphagia in PD. Clinical assessment of dysphagia in PD patients is challenging and often delivers unreliable results. A modified water test assessing maximum swallowing volume is recommended to uncover oropharyngeal dysphagia in PD. PD-specific questionnaires may also be useful to identify patients at risk for swallowing impairment. Fiberoptic endoscopic evaluation of swallowing and videofluoroscopic swallowing study are both considered to be the gold standard for evaluation of PD-related dysphagia. In addition, high-resolution manometry may be a helpful tool. These instrumental methods allow a reliable detection of aspiration events. Furthermore, typical patterns of impairment during the oral, pharyngeal and/or esophageal swallowing phase of PD patients can be identified. Therapy of dysphagia in PD consists of pharmacological interventions and swallowing treatment by speech and language therapists (SLTs). Fluctuating dysphagia with deterioration during the off-state should be treated by optimizing dopaminergic medication. The methods used during swallowing treatment by SLTs shall be selected according to the individual dysphagia pattern of each PD patient. A promising novel method is an intensive training of expiratory muscle strength. Deep brain stimulation does not seem to have a clinical relevant effect on swallowing function in PD. The goal of this review is giving an overview on current stages of epidemiology, pathophysiology, diagnosis, and treatment of PD-associated dysphagia, which might be helpful for neurologists, speech-language therapists, and other clinicians in their daily work with PD patients and associated swallowing difficulties. Furthermore areas with an urgent need for future clinical research are identified.     

Managing Perianal Crohn’s Disease

Perianal Crohn’s Disease (CD) is a significant cause of morbidity in CD patients. Accurate identification of perianal involvement requires advanced imaging techniques in addition to physical exam. Treatment of the disease is aimed at improving both the perianal and intestinal manifestations. Proper treatment depends upon the severity of the disease and combines current medical and surgical therapies to maximize response. The ability to improve perianal disease has grown significantly since the introduction of anti-TNF agents which are now a mainstay of treatment along with antibiotics and immunomodulators. New experimental therapies are limited by lack of research to support their use.     

Whipple’s Disease: A Rare Disease Revisited

Since the original postmortem diagnosis of “intestinal lipodystrophy” by Dr. George H. Whipple in 1907, the complexities of Whipple’s disease have been elucidated through case reports. Universally fatal prior to the advent of antibiotics, Tropheryma whipplei is increasingly recognized as an organism that can be treated only if the clinician seeks to identify it. Whipple’s disease is primarily a gastrointestinal disease manifesting as a malabsorption syndrome, and is detected through endoscopy and intestinal biopsy. Nongastrointestinal manifestations of the disease, although less common, are reported and have aided in its recognition as a multiorgan disease entity. Because of its rarity, treatment recommendations are currently based on observational studies and on one recent prospective study, which outlined induction therapy followed by several months of suppressive maintenance therapy to prevent relapse, which is often characterized by neurologic symptoms.     

Alzheimer’s Disease and Type 2 Diabetes: Multiple Mechanisms Contribute to Interactions

Obesity, metabolic syndrome, and type 2 diabetes (T2D) are related disorders with widespread deleterious effects throughout the body. One important target of damage is the brain. Persons with metabolic disorders are at significantly increased risk for cognitive decline and the development of vascular dementia and Alzheimer’s disease. Our review of available evidence from epidemiologic, clinical, and basic research suggests that neural dysfunction from T2D-related disease results from several underlying mechanisms, including metabolic, inflammatory, vascular, and oxidative changes. The relationships between T2D and neural dysfunction are regulated by several modifiers. We emphasize 2 such modifiers, the genetic risk factor apolipoprotein E and an age-related endocrine change, low testosterone. Both factors are independent risk factors for Alzheimer’s disease that may also cooperatively regulate pathologic interactions between T2D and dementia. Continued elucidation of the links between metabolic disorders and neural dysfunction promises to foster the development of effective therapeutic strategies.