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Primary fibromyalgia syndrome: a critical evaluation of recent criteria developments.

Although nonspecific musculoskeletal aching of nonarticular origin has been described in European literature since the 17th century under such names as muscular rheumatism, the fibrositis syndrome with generalized musculoskeletal aches or stiffness, fatigue, poor sleep, and multiple tender points as a characteristic rheumatologic entity was not described until the 1960s. Smythe first suggested criteria for this condition based on clinical experience in the 1970s. However, the first data-based criteria of "fibrositis" (appropriately called fibromyalgia) was not published until 1981. Since then other criteria have been published, and in 1989 criteria studies on fibromyalgia with appropriate design and controls were reported. This article critically evaluates current criteria developments in fibromyalgia.

Diagnosis, Differential↗

Chronic upper limb pain syndrome (repetitive strain injury) in the Australian workforce: a systematic cross sectional rheumatological study of 229 patients.

The epidemic of chronic upper limb pain is the most important and controversial issue in industrial rheumatology in Australia today. Two hundred and twenty-nine consecutive patients referred with chronic upper limb pain which had been labelled "repetitive strain injury" or "overuse syndrome" were assessed according to a protocol designed to give insight into the questions: Is the pain genuine or falsely reported, i.e., malingering? If genuine is it due to a physical injury, a pain syndrome, or a mixture of both? Twenty-nine patients fulfilled criteria for specific rheumatological diagnoses (fibrositis 15, rotator cuff syndrome 3, rheumatoid arthritis 3, cervical referred pain 3, lateral epicondylitis 2, de Quervain's tenosynovitis 1, carpal tunnel syndrome 1, and psoriatic monoarthritis 1). In the remaining 200 (mean age 37 years, range 19-58, 91.5% female) many different pain patterns and nonspecific associated symptoms were recorded. Eighty-nine percent had greater than or equal to 2 Smythe tender points, 1.5% had 1 tender point, and 9.5% had no tender point. Diffuse pain and greater than 7 tender points is sufficient to diagnose fibrositis, and localized pain and a smaller number of tender points strongly suggests a genuine chronic rheumatic pain syndrome. Stress, personal susceptibility and poor motivation appeared important in some cases. The liberal workers' compensation system, early labeling as repetitive strain injury, and social acceptability appeared important in the development of the epidemic.

Adult↗

Serum angiotensin converting enzyme in sarcoidosis: sensitivity and specificity in diagnosis: correlations with disease activity, duration, extra-thoracic involvement, radiographic type and therapy.

Serum angiotensin converting enzyme (SACE), despite certain limitations, has been found to be a useful adjunct to the diagnosis, assessment of disease activity, and management of sarcoidosis. The spectrofluorimetric assay was used to measure SACE in 50 normal controls, 76 patients with tuberculosis (42 pulmonary, 16 lymphatic and 18 military cases), 20 patients with cryptogenic fibrosing alveolitis, 50 patients with silicosis, three patients with extrinsic allergic alveolitis, 10 patients with Crohn's disease, two patients with Gaucher's disease, and 128 patients with sarcoidosis on 303 occasions (144 during periods judged as clinically active and 189 inactive). Our results show a normal range (mean +/- 2SD) of 19-54 nmol/ml/min. The false positive rate is 2 per cent in normal controls, 9.2 per cent in tuberculosis (38.9 per cent in military but 0 per cent in the other forms), 48 per cent in silicosis, 100 per cent in Gaucher's disease, and 0 per cent in the other diseases. The sensitivity, specificity, positive and negative predictive values were 58.1, 83.8, 83.8 and 58.1 per cent respectively. The sensitivity rose to 85.9 per cent if only those samples taken from patients in whom sarcoidosis was suspected on initial presentation were included, and 92.1 per cent if only those with clinically active sarcoidosis were included. The sensitivity of SACE as a diagnostic test in sarcoidosis is thus influenced by the relative frequency of active and inactive sarcoidosis. The specificity is influenced by the prevalence of military tuberculosis and silicosis, but is uninfluenced by other common varieties of interstitial lung disease such as cryptogenic fibrosing alveolitis and extrinsic allergic alveolitis, or by other non-pulmonary granulomatous disease. There was no correlation of the SACE level with age, sex, population group, associated other illness or duration of sarcoidosis. Statistically, SACE levels were significantly higher in patients with Types II and III chest radiographs as compared to Type I and 0, and also in those with additional clinically evident extra-thoracic disease e.g. in lymph node, eye and especially multiple systems. SACE also reflected clinical activity with levels being statistically significantly greater in those patients assessed as having active disease, although 7.9 per cent of these had normal levels. Our observations indicate that SACE levels correlate well with disease activity longitudinally, both in relation to spontaneous remission and steroid therapy, and are thus helpful in patient management.

Adrenal Cortex Hormones↗

[Pulmonary lesions following bone marrow graft. Study of 35 cases].

Lung biopsy of 35 patients with interstitial pneumonitis following bone marrow transplantation (BMT) have been studied histologically, ultrastructurally and by immunofluorescence. Among infectious diseases, cytomegaloviruses (CMV) are the more frequently found, whereas Pneumocystis carinii infections are more frequently found in immunocompromised hosts without BMT. CMV infections are related to severe chronic graft-versus-host disease in allogenic or mismatched BMT. Hemorrhagic pulmonary oedema and vascular damage might be the consequence of high doses of cyclosporin A or of disseminated intravascular coagulation. Granulomatous and fibrosing lesions corresponded in 2 cases to an eosinophilic pneumonitis and in 11 cases to an "idiopathic" diffuse interstitial pneumonitis. 2 patients had concomitant diffuse lung fibrosis, sclerotic plaques of the skin and Sjögren-like syndrome. The pulmonary and cutaneous scleroses had common features in the types of collagen and in the composition of the infiltrate. Both fibroses might result from a common pathogenic mechanism related to an immunologic conflict between the lymphocytes of the graft and the cells from the host tissues.

Adolescent↗

[Breast-sparing therapy of breast cancer: on the combination of radiation therapy with adjuvant chemotherapy].

From January, 1975 through June, 1986, 426 patients with mammary carcinomas were submitted to primary, breast-preserving therapy at the Gynecological Hospital of the University of Heidelberg. 212 women with a minimum observation time of twelve months fulfilled the criteria of a "typical" treatment: tumor size up to 3 cm, segment/quadrant resection and axillary lymphonodectomy with at least eight lymph nodes removed, radiotherapy of the residual breast with greater than or equal to 45 Gy, in case of histological lymph node manifestation adjuvant hormonal and/or chemotherapy. The average observation time was 38 months, the medium age 48 years. Patients with histological lymph node manifestations were compared with a matched control group of women treated treated by modified radical therapy. According to the error estimation of Kaplan and Meier (1958), no differences were found for local recurrence rate, disease-free survival, and overall survival. Patients treated by organ-preserving therapy with adjuvant chemotherapy were opposed to a matched control group of women treated only by surgical/radiological, organ-preserving therapy. In patients with chemotherapy, the incidence of cutaneous erythema (29% versus 24%), telangiectasia (34% versus 24%), hyperpigmentation (41% versus 34%) showed an upward tendency, but was not significantly increased. There was no difference in the incidence of clinically palpable fibroses (37% versus 42%) and fibroses shown by mammography (54% versus 51%). The frequency of pneumonitis/fibrosis of the retromammary lung area (22% versus 10%) after chemotherapy was two times higher than in the matched control group not treated by chemotherapy.

Antineoplastic Agents↗

[Incidence of collagenoses in patients with lung fibrosis].

In 63 out of 887 patients with alveolitis and pulmonary fibroses was causally found a so-called "collagenosis". A participation of the lung in such diseases is prognostically unfavourable. The changes in the framework of the lungs radiographically, functionally and morphologically cannot be separated from fibroses of other origin. The diagnosis is to be made after exclusion of other noxae and taking into consideration the appearances of the primary disease (clinic, spectrum of organs, special laboratory parameters etc.).

Adolescent↗

[Hypertrophic obstructive cardiomyopathy with extensive myocardial fibrosis: case report with autopsy].

An autopsy case of hypertrophic obstructive cardiomyopathy with extensive myocardial fibrosis is reported in a 43-year-old male. His mother died suddenly at 55. At the age of 39 the patient felt fatigue and feverish sensation followed by dyspnea and palpitation on exertion. He responded to beta-blocker and was discharged on the 51st hospital day. He died suddenly during his work three years and one month after discharge. The heart weighs 700 g. The thickness of the ventricular septum measures up to 3.2 cm, and that of the left ventricular posterior wall 2.2 cm. Subaortic endocardium is moderately thickened. Many patchy fibroses of various sizes and broad linear fibroses are mainly observed in the ventricular septum and in the left ventricular free wall. Microscopic examination shows severe fascicular disarray of hypertrophied myocardial fibers in the ventricular septum and in a part of the left ventricular anterior wall. Pericardial fibrosis, granulation tissue with many capillaries, and slight lymphocytic infiltrate are also noted. These findings suggest that the patient have both congenital hypertrophic cardiomyopathy and myocarditis. There are following possibilities as regards the relation between the two: first, haphazard association of cardiomyopathy with myocarditis; secondly, myocarditis triggered the onset or progression, or both, of cardiomyopathy. He also had liver cirrhosis, probably alcoholic, which appears to accelerate the progression of myocardial disarray and fibrosis.

Adult↗

Smooth muscle within ovarian decidual nodules: a link to leiomyomatosis peritonealis disseminata?

Evidence is presented for the coexistence of smooth muscle and decidual cells in nodules on and within the ovarian tunica albuginea at term. Routine histologic techniques and electron microscopy have been employed in characterizing the morphology of the nodules. Recent literature concerning the frequency of ovarian decidualization during pregnancy is discussed with respect to the possible relationship of such decidualization to the histogenesis of leiomyomatosis peritonealis disseminata (LPD). The hypothesis that LPD may represent "disseminated fibrosing decidua" is discussed in light of finding collagen fibrils, secretory decidual cells, and smooth muscle cells in these nodules. It is concluded that the present case does not represent "fibrosing decidua." The authors agree with others who have proposed that the smooth muscle in ovarian decidua and LPD result from proliferation of stem cells which may reside in the subperitoneal stroma in association with ectopic endometrial stroma and which may respond to the hormones of pregnancy.

Decidua↗

[Changes of pulmonary and bronchial vascularisation in disseminated lung diseases (author's transl)].

Pulmonary and bronchial artery angiographic pictures were evaluated in 14 patients with pulmonary disseminations. Diffuse fibrosing alveolitis was present in 4 patients, in 4 other patients secondary pulmonary fibrosis and in 6 patients pulmonary sarcoidosis. Most changes of pulmonary vascularisation were found in the lobular and terminal branches of the pulmonary artery. They consisted in their narrowing and even occlusion. Different degrees of the reduction of pulmonary perfusion could be observed. The most morbid alterations were in fibrosing alveolitis. They were less pronounced in cases of secondary fibrosis, showing a localized character, and in sarcoidosis in which the upper pulmonary parts were involved. Bronchial artery vascularisation was showing hypervascularisation of various degrees. Increased and dilated vessels strongly winding and even reticularly arranged could be regularly found. These alterations were very pronounced in patients with sarcoidosis. After transgressing of the pathological process to the state of fibrosis the vessels were showing a striped course presenting a greater number of broncho-pulmonary anastomoses.

Adult↗

[A rare tumor: benign sclerosing pneumocytoma with an intrascissural development].

In 1956, LIEBOW and HUBBEL defined pulmonary fibrosing haemangioma as a marked vascular proliferation with a marked tendency to fibrosis, papillary vegetations, extensive histiocytic infiltration and haemorrhages at various stages of organisation. More than 70 cases have been reported since. This lesion occurs most often in the middle aged woman. It is usually asymptomatic or may present as haemoptysis. Radiologically, it takes the form of a well-limited round homogeneous opacity and the prognosis is excellent after excision. The case reported here fell fully within this context and had the special feature of intrascissural tumour development resulting in an unusual radiological appearance which initially suggested a diagnosis of encysted pleural effusion. However the solid nature of the opacity being confirmed by CT scan, it was excised surgically. Its nature was revealed by histological examination. A detailed review of the literature is undertaken and changes in the histopathological concept of this type of lesion discussed. Previously classified amongst inflammatory pseudo-tumours, it is now considered to be a tumour proliferation which, on the basis of current data from electron microscopy and histochemistry, is felt by some to be of vascular origin but by the majority to be of epithelial origin, apparently developing from immature type II pneumocytes. The name "benign fibrosing pneumocytoma" suggested by CHAN would seem now more appropriate.

Female↗

[Liver echinococcosis].

On the basis of the experience with surgical treatment of 168 cases of hepatic echinococcosis in Algeria the author has concluded that such cases need an intense therapy both in pre- and postoperative period. To prevent from the recurrence of the disease it is sufficient to process the hydatid cyst fibrosal tunic with a tampon moistened with 5% formaline or 1% tripaflavine solution. The excision of the fibrosal tunic extended down to the cyst "bottom", followed by the suturing of the capsule stump to the liver wound margins, or primary suturing, before the drainage is started, in cases of a narrow wound cavity, result in 98.8% of recovery.

Adolescent↗

Physiological variables and mortality in patients with various categories of chronic respiratory disease.

As an introduction to a prospective study on the complex non-invasive estimation of pulmonary arterial pressure, the mortality experience of 834 patients with chronic respiratory disease was analysed historically. In patients with chronic obstructive lung disease, chronic fibrosing alveolitis and pulmonary tuberculosis, the relationships between mortality and values of ten physiological variables were examined. The most important variable as regards mortality in chronic obstructive lung disease was FEV1.0; after controlling for FEV1.0, pulmonary arterial pressure, arterial oxygen and carbon dioxide pressures were all strongly related to mortality. By contrast, in fibrosing alveolitis, values of FEV1.0 had little relation to mortality, and pulmonary arterial pressure was the most important variable, followed by FVC and FEV/VC. In patients with tuberculosis, arterial oxygen and pulmonary arterial pressure had the strongest relationship with mortality. Pulmonary arterial pressure was therefore strongly related to mortality in all three conditions; FEV1.0 had its highest predictive power in chronic obstructive lung disease and arterial oxygen saturation in tuberculosis. In chronic obstructive lung disease, pulmonary arterial pressure had a stronger influence in relation to mortality of younger than of older patients.

Blood Pressure↗

Fibromatosis of the oral cavity.

Nine cases of fibromatosis occurring in or near the oral cavity are presented here. They are classified as nodular fasciitis (3 cases), aggressive infantile fibromatosis (2 cases), fibrosing myositis (1 case) and fibromatosis (1 case). Of the 5 cases of nodular fasciitis, 3 occurred from the fascia of the masticatory muscle and 2 from the submucous fascia of the palate and tongue. The aggressive infantile fibromatosis showed a prominent infiltrating growth and an alarming histologic appearance. One of the 2 cases showed a recurrence, but the postoperative course of both cases was uneventful for 13 years and 10 years, respectively. The fibrosing myositis is an unusual fibromatosis of the muscle, which arose within the tongue muscle. The lesion was benign and can be cured by conservative surgery.

Adult↗

[Pulmonary biopsy in interstitial pneumopathies].

1119 pulmonary biopsies (two thirds open) have been performed in 108 patients with signs of diffuse interstitial disease of the lung. The results showed 29 cases of fibrosing alveolitis, 4 of granulomatous alveolitis, 8 of diffuse interstitial fibrosis, 12 of eosinophilic granuloma, 12 of pneumoconiosis, 16 of sarcoidosis, and 38 with other pulmonary lesions. Biopsy via thoracotomy is the best diagnostic technique, with a 97.4% yield; with transparietal biopsies the yield is 81% and with transbronchial biopsies 60%. Histology permits exact diagnosis in all pneumopathies except fibrosing alveolitis. In this case the pathologist guides the clinician's investigations towards a search for an allergic etiology.

Alveolitis, Extrinsic Allergic↗

[Physical therapy and rheumatism of soft tissues].

Soft tissue rheumatism (extraarticular rheumatism) is a group of common disorders found separately as independent disorders or as a symptom of systemic joint or spine diseases. The most frequent soft tissue rheumatisms are tendinopathies, rheumatism of muscles with myofascial pains, fibrositis of subcutaneous tissue and, finally, bursitis and inflammations of tendon sheaths. The commonest therapeutic procedures are non-steroidal anatirheumatic drugs, local infiltrations of corticosteroids, and physiotherapy. In the acute stages cold packs are the best physical measure against subjective pain. In subacute and chronic cases the pain in subcutaneous tissues can be lessened by connective tissue massage and underwater jet massage, muscle pain by heat and active exercises, and tendinopathies by ultrasonics and electrotherapy but very seldom by heat. In chronic bursitis and tendovaginitis, iontophoresis with potassium iodine may be helpful. Reflex pains in muscles are due to lesions of the spine and should therefore be treated by spine extension and manipulations. Every pain in soft tissue has one source in the anatomic lesion of the tissue and the second in psychogenic disorders. The latter must be sought when soft tissue pains are found all over the body on the lines of a generalized fibrositis syndrome.

Adrenal Cortex Hormones↗

[Treatment of diffuse lung fibrosis with D-penicillamine].

Pulmonary fibroses which are regarded as reactive final stage of a great number of pulmonary diseases apart from their unfavourable prognosis may also therapeutically be scarcely influenced. With D-penicillamine a remedy was found which first intervened directly into the collagenic metabolism. Mechanisms of action, therapy indications and side-effects are compiled taking into consideration a broad survey of literature. Together with the casuistic description of three cases of treatment of a pulmonary fibrosis with D-penicillamine is reported on won experiences. As criterion of the therapeutic effectivity the general condition, the X-ray findings, above all, however, functional parameters of the lungs are regarded. When the pros and cons of the expenditure and the high frequency of side-effects of D-penicillamine compared with the changing therapeutic results with this medicament are considered must also thought of the fact of an otherwise not existing possibility of an effective influencibility of the pulmonary fibrosis. Actual tendencies and experiments for the improvement of the fibrose therapy are discussed.

Adult↗

Tryptophan metabolism in chronic inflammatory lung disease.

Induction of indoleamine 2,3-dioxygenase (IDO), an enzyme expressed by mononuclear phagocytes and some fibroblast cell lines in response to interferon-gamma, leads to enhanced degradation of tryptophan to kynurenine. Because inflammatory lung diseases are generally associated with activation of pulmonary macrophages, we investigated tryptophan metabolism in patients with interstitial lung disease by measuring circulating levels of tryptophan and kynurenine in peripheral blood and by measuring the IDO activity of bronchoalveolar cells. IDO activities were increased for bronchoalveolar lavage (BAL) cells obtained from patients with interstitial lung disease (115.4 +/- 30.4, n = 37) when compared with BAL cells from normal subjects (15.2 +/- 7.4, n = 14; p < 0.05), and messenger RNA for IDO was present in BAL cells from patients with interstitial disease but was not present in BAL cells from normal volunteer subjects. Patients with inflammatory lung disease also had decreased tryptophan and increased kynurenine concentrations in serum. The ratio of serum tryptophan levels to serum kynurenine levels was significantly depressed for patients with idiopathic pulmonary fibrosis (18.4 +/- 1.7, n = 29; p < 0.0001), patients with fibrosing alveolitis associated with collagen vascular disease (13.1 +/- 1.6, n = 18; p < 0.0001), or patients with sarcoidosis (21.0 +/- 1.1, n = 50; p < 0.0001), as compared with the ratio for normal subjects (31.8 +/- 2.3, n = 18). Patients with fibrotic disease had the highest levels of BAL cell IDO activity, and patients with collagen vascular disease associated fibrosing alveolitis had the most depressed levels of serum tryptophan and the greatest elevations in serum kynurenine. Measurement of tryptophan and kynurenine concentrations in serum may provide a useful measure of disease activity in chronic inflammatory parenchymal lung diseases such as sarcoidosis and idiopathic pulmonary fibrosis.

Actins↗

Idiopathic sclerosing inflammation of the orbit: immunohistologic analysis and comparison with retroperitoneal fibrosis.

Idiopathic sclerosing inflammation of the orbit is clinically characterized by an insidious, chronic and progressive fibrosing process damaging orbital structures through entrapment and mass effect. Histologically, desmoplasia and a sparse infiltrate of lymphocytes, histiocytes, plasma cells, and occasional neutrophils and eosinophils are seen. An immune pathogenesis is suspected but presently poorly understood. To characterize the inflammatory infiltrate and to compare orbital and other inflammatory fibrosing lesions, immunoperoxidase studies using the streptavidin method were performed on 16 formalin or Bouins' fixed, paraffin-embedded orbital biopsy specimens and six specimens of retroperitoneal fibrosis. Positive staining of orbital tissue occurred as follows: T-cells (UCHL-1) 94% of cases, B-cells (L26) 40%, tissue macrophages (KP-1) 56%, HLA Dr positive antigen presenting cells and activated T-cells (LN3) 44%, and immunoglobulins (kappa, 80%; lambda, 63%, IgG, 73%, IgA, 44% and IgM, 31%). Results were strikingly similar for retroperitoneal fibrosis. These findings imply a cell mediated pathogenesis in idiopathic sclerosing inflammation of the orbit that is similar to retroperitoneal fibrosis and suggest therapeutic potential for agents modifying this facet of the immune system.

Humans↗