Systemic inflammatory response syndrome and acute neurological disease.

Systemic inflammatory response syndrome is a common clinical entity often presenting as a complication of neurological disease or trauma. Clear diagnostic criteria exist. Induced pathological mechanisms include both immunological and endothelial dysfunction, and coagulopathy, which may lead to multiple organ failure and significant morbidity. Possible therapeutic mechanisms are discussed, but this complex syndrome is poorly understood and successful treatment may depend on further research into control mechanisms.     

Pulmonary chronic thromboembolic disease

Persistent thrombotic lesions are common in patients with pulmonary embolism. These lesions occur on a clinical spectrum, ranging from an asymptomatic course with complete functional recovery to chronic thromboembolic pulmonary hypertension. The concept of chronic thromboembolic disease has emerged in recent years to describe a subgroup of patients with persistent thrombotic lesions who have symptoms on exertion and pulmonary vascular dysfunction, but no pulmonary hypertension at rest. The prevalence of this entity is unknown and the criteria for diagnosing it are not defined. The aim of this article is to analyze post-pulmonary embolism sequelae and review existing evidence on chronic thromboembolic disease, with special emphasis on its diagnosis and therapeutic approach.     

Clinical features of polymyalgia rheumatica and giant cell arteritis

Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) are inflammatory diseases that typically affect white individuals >50 years. Women are affected ～2-3 times more often than men. PMR and GCA occur together more frequently than expected by chance. The main symptoms of PMR are pain and stiffness in the shoulders, and often in the neck and pelvic girdle. Imaging studies reveal inflammation of joints and bursae of the affected areas. GCA is a large-vessel and medium-vessel arteritis predominantly involving the branches of the aortic arch. The typical clinical manifestations of GCA are new headache, jaw claudication and visual loss. PMR and GCA usually remit within 6 months to 2 years from disease onset. Some patients, however, have a relapsing course and might require long-standing treatment. Diagnosis of PMR and GCA is based on clinical features and elevated levels of inflammatory markers. Temporal artery biopsy remains the gold standard to support the diagnosis of GCA; imaging studies are useful to delineate large-vessel involvement in GCA. Glucocorticoids remain the cornerstone of treatment of both PMR and GCA, but patients with GCA require higher doses. Synthetic immunosuppressive drugs also have a role in disease management, whereas the role of biologic agents is currently unclear.     

Classification criteria for spondyloarthropathies

Spondyloarthropathies (SpA) are a group of inflammatory arthritis which consist of ankylosing spondylitis (AS), reactive arthritis, arthritis/spondylitis associated with psoriasis (PsA), and arthritis/spondylitis associated with inflammatory bowel diseases. It is now more important than ever to diagnose and treat SpA early. New therapeutic agents including blockers of tumor necrosis factor have yielded tremendous responses not only in advanced disease but also in the early stages of the disease. Sacroiliitis on conventional radiography is the result of structural changes which may appear late in the disease process. However, magnetic resonance imaging (MRI) can visualize active inflammation at sacroiliac joints and spine in recent onset disease. The modified New York criteria, the European Spondyloarthropathy Study Group criteria and the Amor criteria do not include advanced imaging techniques like MRI which is very sensitive to the early Inflammatory changes. Assessment of SpondyloArthritis international Society has defined MRI methods for the assessment of sacroiliac joints and spine, criteria for inflammatory back pain and developed new criteria for classification of axial and peripheral spondyloarthritis. These new criteria are intended to be used for patients with SpA at the very early stage of their disease. Also, classification of psoriatic arthritis study group developed criteria for the classification of PsA. The widespread use of these criteria in clinical trials will provide evidence for a better definition of early disease and recognize many patients who may further develop classical AS or PsA. These efforts will guide therapeutic trials of potent drugs like biological agents in the early stage of these diseases.     

The clinical value of the palmomental reflex

Summary The age specific incidence of the palmomental reflex (PMR) has been investigated in persons without any former or present evidence of a neurological or mental involvement. Also excluded were persons with hypertension, diabetes mellitus, thyroid dysfunction, alcohol abuse and other diseases as these could effect the central or peripheral nervous system. From the newborn period up to the age of 20 years the incidence of the PMR was between 3,3% and 20%, the differences being statistically insignificant.After the age of 20 years the incidence of the PMR rises with increasing age, the rise being approximately 10% per decade.There was no asymmetry of the PMR in persons without affection of the nervous system and who met the above mentioned criteria.Since a symmetrical PMR can be found in a considerable percentage of persons with no indication of a neurological or mental involvement, it should not be considered as a pathological sign.An asymmetrical PMR was found in 20 persons who did not meet the above mentioned criteria. In 5 out of these 20 persons additional neurological signs could be detected. 9 patients had histories of brain trauma, meningitis, cerebrovascular disease and polyneuropathy. 9 others were suffering from severe cardiovascular disease, carcinoma and alcohol abuse. In only one patient, although presenting with some neurological signs, no relevant history could be detected.An asymmetrical PMR, therefore, must be considered as a discrete indication of an involvement, either of the central or the peripheral nervous system. The PMR has no certain localizing significance.     

Neurogenic inflammation and chronic pelvic pain

 Chronic pelvic pain is a puzzling disease entity. The pathophysiological mechanisms of chronic pelvic pain are not clear and current treatment strategies are often not successful, leaving patients as well as health care providers frustrated. In a subgroup of patients with chronic pelvic pain (e.g., interstitial cystitis, irritable bowel syndrome, vulvar vestibulitis, prostatodynia/prostatitis, and loin pain/hematuria syndrome) inflammatory changes are observed, for which no etiology has been identified. These inflammatory changes might be due to neurogenic inflammation. Applying the concept of neurogenic inflammation to chronic pelvic pain provides new insights into the pathophysiological mechanisms of these pain syndromes, makes it possible to account for the heterogeneity and variability observed in the clinical presentation, and might lead to the development of novel therapies.     

Dermatomyositis and polymyositis

Opinion statement Dermatomyositis (DM) and polymyositis (PM) are idiopathic inflammatory myopathies characterized by proximal greater than distal muscle weakness, elevated serum creatine kinase levels, electrophysiologic abnormalities, and inflammation on muscle biopsy. Clinically and electrophysiologically, DM and PM appear very similar, and muscle biopsy is the gold standard for diagnosis. Much of the PM literature based the diagnosis on Bohan and Peter’s criteria, which is now obsolete given the advances of immunopathology. As diagnostic criteria for the inflammatory myopathies have been refined, it has become apparent that PM is much less common than previously thought, and, in fact, is probably quite rare. More recent literature, using strict histopathologic criteria for diagnosis of PM, has brought into question previously reported associations. Because of this, the clinical entity of PM is poorly defined. The exact incidence of each is unknown because previous epidemiologic studies often grouped them together, but overall the annual incidence of the inflammatory myopathies is approximately one in 100,000. DM and PM respond to immunomodulating therapies. High-dose oral prednisone is generally accepted first-line therapy. In patients who do not respond adequately to prednisone alone, or in whom prednisone cannot be weaned, methotrexate or azathioprine can be added. In the authors’ experience, methotrexate works faster and is more effective than azathioprine. However, because of the increased risk of interstitial lung disease with methotrexate, the authors avoid this in patients with anti-Jo-1 antibodies and, obviously, in patients who already have pulmonary disease. If patients do not respond adequately to the combination of prednisone and methotrexate or azathioprine, a trial of intravenous immunoglobulin is administered.     

Crescentic glomerulonephritis in hyper IgD syndrome

 The hyperimmunoglobulinemia D syndrome (HIDS) is a well-defined entity resembling familial Mediterranean fever. HIDS is a systemic inflammatory disease associated with stimulation of T-cell-mediated immunity. These patients are at low risk for amyloidosis and are not known to develop nephropathy. We report a boy of Mediterranean ancestry who exhibited typical HIDS and end-stage renal failure. Kidney biopsy revealed pauci-immune crescentic glomerulonephritis (cGN). We hypothesized that the glomerular involvement was secondary to the cytokine network activation observed in HIDS. Thus, cGN should be considered as part of the syndrome, and kidney biopsy should be performed early in the course of the renal disease in patients with HIDS. Received: 2 February 1998 / Revised: 30 June 1998 / Accepted: 2 July 1998     

Thymic Carcinoid Associated With Multiple Endocrine Neoplasia Syndrome Type I

Thymic carcinoids are a rare entity that may be associated with endocrine diseases like Cushing's syndrome or multiple endocrine neoplasia syndrome type I (MEN1). These tumors represent 4% of anterior mediastinal tumors and are characterized by their very aggressive behavior.We present the case of a patient with a previous MEN 1 diagnosis in whom, during the follow up of his disease, a thoracic image compatible with thymic carcinoid was detected. After an extended thymectomy that included peri-thymic fat resection, the clinical diagnosis was confirmed. A follow-up examination 14 months later revealed a suspicious lesion that suggested local recurrence, therefore the patient was reoperated on. The pathology report of this surgery indicated post-radiation fibrosis.Likewise, we present a review of the current diagnostic and therapeutic management of patients with MEN1 syndrome who are diagnosed with thymic carcinoid.     

Depression and depressive symptoms in patients with polymyalgia rheumatica: discussion points,grey areas and unmet needs emerging from a systematic review of published literature

ObjectivesIn 1979, Bird et al. proposed depression as a diagnostic criterion for polymyalgia rheumatica (PMR). More recently, the significance of depression in PMR patients has been re-proposed, , and some researchers have suggested that PMR may increase the risk of depression. The aim of our article is to evaluate the relationship between PMR and depression.Material and methodsSystematic literature searches were performed on 19^th and 20^th May 2020 based on Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines. The search was restricted to all studies and case reports with English abstract, published in any language, since 1979 (when depression was first proposed as a diagnostic criterion for PMR) describing the association of PMR with depression. Exclusion criteria were as follows: reviews, conference abstracts, comments, non-original articles; and articles discussing giant cell arteritis (GCA) and PMR when data and observations for the two conditions were not clearly subdivided.ResultsThe initial search yielded 812 papers, of which 115 duplicates were removed. A total of 697 articles had a first screening and 506 were excluded based on title and abstract reviews; 117 articles underwent full-length scrutiny, and 99 full-text articles were excluded because they did not meet the inclusion and exclusion criteria (reviews and comments = 58; articles with outcome of interest not reported = 34; low-quality articles = 7). At least, 18 articles were included in this review.ConclusionsThe review did not find any studies that clarified the prevalence rates of depression in patients with PMR. Furthermore, the studies reviewed did not offer any clarity as to whether patients suffered from just depressive symptoms or clinical depression, and that accepted diagnostic criteria for depression had not been employed, indicating that a robust method for diagnosing depression had not been employed. Collaboration of different professionals should be improved through shared guidelines.     

Polymyalgia rheumatica: diagnosis and treatment

Polymyalgia rheumatica (PMR) typically manifests as inflammatory pain in the shoulder and/or pelvic girdles in a patient over 50 years of age. This condition was long underrecognized and therefore underdiagnosed. Today, however, overdiagnosis may occur. Physicians must be aware that many conditions may simulate PMR, including diseases that carry a grim prognosis or require urgent treatment. PMR may be the first manifestation of giant cell arteritis, and a painstaking search for other signs is mandatory. PMR may inaugurate other rheumatologic diseases such as rheumatoid arthritis, RS3PE syndrome, spondyloarthropathy, systemic lupus erythematosus (SLE), myopathy, vasculitis, and chondrocalcinosis. Finally, PMR may be the first manifestation of an endocrine disorder, a malignancy, or an infection. Failure to respond to glucocorticoid therapy should suggest giant cell arteritis, malignant disease, or infection. Ultrasonography may assist in the diagnosis by showing bilateral subdeltoid bursitis. Glucocorticoids are the mainstay of the treatment of PMR. Although the optimal starting dosage and tapering schedule are not agreed on, a low starting dosage and slow tapering may decrease the relapse rate. Methotrexate is probably useful when glucocorticoid dependency develops. In contrast, TNF-alpha antagonists are probably ineffective.     

Thoracic outlet syndrome. Thoracic surgery perspective.

We have attempted throughout this review to identify the issues surrounding thoracic outlet syndrome as well as to highlight their origins. It should be clear that many aspects of TOS remain controversial from the definition of the entity through pathogenesis, diagnosis, and treatment. The conflicts surrounding TOS are underlined most poignantly in the many letters to the editor of the New England Journal of Medicine in response to Urschel's 1972 publication. It is incumbent upon those of us who treat patients with TOS to dispel the ignorance surrounding this syndrome with astute, accurate, and reproducible observations. We must clearly define TOS as a clinical entity such that we may analyze the characteristics of the patients we treat. We must continue to search for innovative and specific diagnostic criteria. We must quantitatively and reproducibly measure subjective end points of pain severity and quality of life. The use of these methods will provide yardsticks for therapeutic success and act as determinants for the natural history of TOS. The objectives of treatment will remain the alleviation of symptoms and the restoration of function. We have applied these principles to the formulation of a protocol in which we record, in a prospective manner, both routine and innovative clinical parameters. With quantification of subjective end points, we may be able to correlate clinical presentation with outcome. We also may be able to define with some accuracy this entity we call thoracic outlet syndrome.     

Interstitial cystitis. An important differential diagnosis

Interstitial cystitis (IC) is a chronic, debilitating inflammatory disease of the urinary bladder, characterized by chronic pelvic pain, urgency/frequency symptoms, and decrease of the functional bladder capacity. Both the etiology and pathogenesis of the condition that predominantly affects women are still not fully understood. Thus, a causal therapeutic approach still does not exist. IC remains a strict diagnosis of exclusion; distinct histopathological findings within the bladder wall are frequently found, although they are not pathognomonic. The diagnostic criteria elaborated by the NIH are helpful in clinical research; their dogmatic utilization will however lead to an underdiagnosis of IC in 60% of the patients. IC-specific symptom questionnaires and validated symptom scales help the physician to assess the therapeutic response during the follow-up of the patients.     

Interstitielle Zystitis

Interstitial cystitis (IC) is a chronic, debilitating inflammatory disease of the urinary bladder, characterized by chronic pelvic pain, urgency/frequency symptoms, and decrease of the functional bladder capacity. Both the etiology and pathogenesis of the condition that predominantly affects women are still not fully understood. Thus, a causal therapeutic approach still does not exist. IC remains a strict diagnosis of exclusion; distinct histopathological findings within the bladder wall are frequently found, although they are not pathognomonic. The diagnostic criteria elaborated by the NIH are helpful in clinical research; their dogmatic utilization will however lead to an underdiagnosis of IC in 60% of the patients. IC-specific symptom questionnaires and validated symptom scales help the physician to assess the therapeutic response during the follow-up of the patients.     

Physikalische Therapie im Kontext orthop?discher Rheumatologie

In this review only some of the physical therapeutic options for treating chronic inflammatory diseases are discussed. These include a wide variety of procedures and should not exclusively be assessed using the criteria of evidence-based medicine because in most studies there was no blinding or for ethical reasons no placebo group was included. Nevertheless, these treatment options are quite essential as part of a multimodal treatment concept for patients with inflammatory joint diseases. The increasing interest in adjuvant therapeutic options emphasizes the need for further well designed studies concerning the effectiveness of physical therapy. Dynamic exercise is closely integrated into the treatment strategy for rheumatoid arthritis. In addition to a conditioning stimulus to joints and cartilage it is known that physical therapy is useful in preventing mechanisms of disease chronification. Locally applied and whole body cryotherapy leads to muscular relaxation resulting in a more effective treatment intensity of subsequent exercise. With extracorporeal shockwave therapy (ESWT) a new promising therapeutic approach is available. However, the evidence level is still weak when used for patients with rheumatoid arthritis. Locally applied steroid injections still have a significant value when treating inflammatory synovial conditions.     

Cell culture approaches to the analysis of glomerular inflammation

In this paper, we have attempted to provide an overview of the methods and findings of a large number of investigators who have dealt with an analysis of the glomerular inflammatory response using tissue culture techniques. These observations represent only a beginning. With the growing interest in this aspect of kidney disease, it is to anticipated that many further advancements in the understanding of the cell biology of the glomerulus are forthcoming. The translation of this fundamental information into new diagnostic and therapeutic modalities is an exciting challenge to investigative nephrology.     

Diagnosis of Moyamoya Disease: International Standard and Regional Differences

Moyamoya disease is a chronic, occlusive cerebrovascular disease with unknown etiology characterized by bilateral steno-occlusive changes at the terminal portion of the internal carotid artery and an abnormal vascular network at the base of the brain. These diagnostic criteria of the moyamoya disease, stated by the Research Committee on Spontaneous Occlusion of the Circle of Willis (moyamoya disease) in Japan, are well established and generally accepted as the definition of this rare entity. On the contrary to the diagnosis of definitive moyamoya disease, there is some confusion in the terminology and understanding of quasi-moyamoya disease; moyamoya disease in association with various disease entities, such as atherosclerosis, autoimmune diseases, Down syndrome, etc. Although the clinical management is not affected by these semantic distinctions, terminological confusion may interfere with the international collaboration of the clinical investigation of these rare conditions. In this article, we sought to review the international standard and regional differences in the diagnosis of moyamoya disease and quasi-moyamoya disease.     

Acute bacterial prostatitis and chronic prostatitis/chronic pelvic pain syndrome: andrological implications

There is a consensus on the diagnostic management of bacterial prostatitis (acute and chronic). In chronic prostatitis/chronic pelvic pain syndrome (CP/CPPS) the diagnostic approach remains unclear, because inflammatory and noninflammatory CP/CPPS might be one entity with varying findings over time. The WHO definition of male accessory gland infection does not differentiate between prostatitis, epididymitis, and other inflammatory alterations of the urethral compartment. The definition therefore cannot be further accepted as a rational tool for the diagnosis of prostatitis and related diseases in urological andrology. Therapy in infectious prostatitis is standardised and antibiotics are the primary agents. Andrological implications are well defined, side-effects are minimal. CP/CPPS therapy has the goal to reduce pelvic pain. However, treatment regimens are not as standardised. Andrological side-effects are well defined and mainly due to the functional background of these agents.     

Das Prostatitissyndrom Ejakulatveränderungen und Auswirkungen auf die Fertilität

Inflammation and infections of the male reproductive tract represent an important factor in male infertility that is potentially responsive to successful therapy. A standardized diagnostic procedure is a prerequisite for the exact evaluation of alterations of ejaculate specimens, especially in cases of the different forms of the prostatitis syndrome. Several alterations in ejaculate due to inflammation and infection have been identified that have a negative impact on the quality of sperm. Besides the influence of leuko- and bacteriospermia, the effect of inflammatory mediators, such as cytokines and reactive oxygen species (ROS), are being discussed as relevant pathomechanisms. These parameters just recently were incorporated into the conventional range of diagnostic criteria. Clinical studies that investigate the quality of ejaculate in the different forms of the prostatitis syndrome yielded contradictory results. Only antimicrobial therapy has been sufficiently described as a therapeutic option. In the future, it will be decisive to include novel functional and molecular parameters to define an interaction of urogenital infection and male infertility.