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Elevated serum galactosylhydroxylysyl glucosyltransferase, a collagen synthesis marker, in fibrosing lung diseases.

The activity of galactosylhydroxylysyl glucosyltransferase, an enzyme catalyzing collagen biosynthesis, was measured in the sera of 101 patients with various pulmonary diseases to study whether detectable enzyme amounts are liberated into the serum from the lung tissue, and whether this is associated with the development of lung fibrosis. Increased serum galactosylhydroxylysyl glucosyltransferase activity was found in all the patients with progressive pulmonary fibrosis and in about half of the patients with acute stages of farmer's lung and infectious pneumonia. In one third of the patients with stage I sarcoidosis the serum enzyme activity was slightly increased, whereas in bronchial asthma and chronic bronchitis the values were mostly within the normal range. In conclusion, elevated serum enzyme activity was demonstrated in connection with those respiratory diseases in which pulmonary fibrosis was already verifiable or relatively often develops later. Measurements of serum galactosylhydroxylysyl glucosyltransferase may, thus, be useful in evaluating actual lung collagen synthesis in human pulmonary diseases.

Adolescent↗

Primary fibromyalgia (fibrositis): clinical study of 50 patients with matched normal controls.

Detailed clinical study of 50 patients with primary fibromyalgia and 50 normal matched controls has shown a characteristic syndrome. Primary fibromyalgia patients are usually females, aged 25-40 yr, who complain of diffuse musculoskeletal aches, pains or stiffness associated with tiredness, anxiety, poor sleep, headaches, irritable bowel syndrome, subjective swelling in the articular and periarticular areas and numbness. Physical examination is characterized by presence of multiple tender points at specific sites and absence of joint swelling. Symptoms are influenced by weather and activities, as well as by time of day(worse in the morning and the evening). In contrast, symptoms of psychogenic rheumatism patients have little fluctuation, if any, and are modulated by emotional rather than physical factors. In psychogenic rheumatism, there is diffuse tenderness rather than tender points at specific sites. Laboratory tests and roentgenologic findings in primary fibromyalgia are normal or negative. Primary fibromyalgia should be suspected by the presence of its own characteristic features, and not diagnosed just by the absence of other recognizable conditions. This study has also shown that primary fibromyalgia is a poorly recognized condition. Patients were usually seen by many physicians who failed to provide a definite diagnosis despite frequent unnecessary investigations. A guideline for diagnosis of primary fibromyalgia, based upon our observations, is suggested. Management is usually gratifying in these frustrated patients. The most important aspects are a definite diagnosis, explanation of the various possible mechanisms responsible for the symptoms, and reassurance regarding the benign nature of this condition. A combination of reassurance, nonsteroidal antiinflammatory drugs, good sleep, local tender point injections, and various modes of physical therapy is successful in most cases.

Adolescent↗

The concept of primary fibromyalgia (fibrositis): clinical value, relation and significance to other chronic musculoskeletal pain syndromes.

PFS, MPS and TMPDS can be identified using positive diagnostic criteria among patients presenting with chronic pain. A directed rather than exhaustive search for organic diseases known to coexist with these syndromes is usually all that is necessary. Criteria are presently empirical but do identify homogeneous populations of patients for study and treatment. Some patients, however, provide examples of overlap and it may be useful to think of CMPS in terms of the Venn diagram depicted in Fig. 2. In this report we have attempted an initial classification for a group of common and perplexing chronic pain disorders of the musculoskeletal system which at present have no identifiable cause. Previous investigations have been hampered by erroneous pathological concepts, heterogeneous patient populations and poor study design particularly with respect to treatment modalities. We hope that this classification, while empirical, will lead to needed epidemiological studies outlining the similarities and differences between these clinically observable and different musculoskeletal syndromes. We hope, further, that it will foster cooperation between different medical disciplines so that clinical biases might be tested in light of current concepts of the scientific method.

Bone Diseases↗

Scintigraphy compared to other imaging modalities in benign superior vena caval obstruction accompanying fibrosing mediastinitis.

Evaluation of a 50-year-old man with benign but insidious development of chronic superior vena caval obstruction secondary to old pulmonary histoplasmosis is presented. The radiologic evaluation was done in stages to exclude involvement of other disease. Nuclear scintigraphy revealed the superior vena caval obstruction. Anatomical features are compared to dynamic findings using contrast venography, computed tomography, and magnetic resonance imaging to differentiate pulmonary vascular compression from neoplasm and pulmonary embolism. The combination of modalities reveals the character and extent of pathology without extensive interventional procedures.

Fibrosis↗