Air in the oesophagus: A sign of oesophageal involvement in systemic sclerosis

Summary An air oesophagogram, defined as a column of air involving the entire oesophagus, seen on a lateral chest X-ray was observed in 6 (20%) of 30 consecutive patients with systemic sclerosis (SSc) but in none of the controls. The presence of this sign was unrelated to the clinical subset of SSc and to age but was associated with the symptom of regurgitation.     

Diffuse alveolar damage: uncommon manifestation of pulmonary involvement in patients with connective tissue diseases

BACKGROUND: Diffuse alveolar damage (DAD) is a relatively common finding on surgical lung biopsy and can result from a variety of causes. METHODS: We studied nine consecutive patients with connective tissue disease (CTD) and DAD diagnosed on surgical lung biopsy to examine this association and clinical implications. RESULTS: The median age was 63 years (range, 35 to 76 years), and seven of the patients were women (78%). Underlying CTDs included rheumatoid arthritis in five patients, polymyositis in two patients, and one patient each with systemic sclerosis and mixed CTD. In seven patients (78%), CTD had been diagnosed before the onset of DAD; six of these patients had a preexisting interstitial lung disease (ILD) related to their CTD. DAD was the presenting manifestation leading to a new CTD diagnosis in two patients (22%). CT of the chest revealed ground-glass opacities and/or consolidation bilaterally with or without honeycombing. In all patients, surgical lung biopsy revealed DAD for which no cause could be identified other than the underlying CTD. Seven patients (78%) were receiving mechanical ventilatory support at the time of the surgical lung biopsy. Four patients (44%) survived to hospital discharge and included one patient with preexisting ILD and all three patients without chronic ILD. CONCLUSION: We conclude that DAD can complicate the clinical course of patients with CTD-related chronic ILD, or can occasionally occur as a presenting manifestation of CTDs. When DAD occurs in patients with CTDs, the outcome appears to be worse for those with preexisting chronic ILD compared to those without ILD.     

Ocular involvement in connective tissue disorders

Connective tissue disorders have well-known ocular associations that may be presenting features of the disease or occur as complications during the course of the disorder. Keratoconjunctivitis sicca, keratitis, scleritis, uveitis, retinal vasculitis, choroidopathy, and their associations with rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, psoriatic arthritis, juvenile idiopathic arthritis, and polymyositis are reviewed.     

心肺功能运动试验对结缔组织病肺损害的评价

目的 探讨运动心肺功能测定在结缔组织病亚临床肺损害的早期诊断价值。方法 采用Ｊａｅｇｅｒ公司产电闸功率自行车,依据ｒａｍｐ－ｐａｔｔｅｒｎ方案,每２分钟连续递增运动负荷２５Ｗ,期间采用心电图监测心率和节律。     

Pulmonary involvement in mixed connective tissue disease

To determine the frequency of pulmonary function abnormalities in mixed connective tissue disease (MCTD), we studied 16 consecutive patients. Spirometry, total lung capacity (TLC), diffusing capacity (Dco), static lung compliance (Cst), and tests of respiratory muscle function (peak inspiratory pressure [PIP] and the maximum change in transdiaphragmatic pressure [delta PDI] during a diaphragmatic Müeller maneuver) were obtained. Airway resistance (RAW) and frequency dependence of compliance were also measured. Static lung compliance was less than 0.2 L/cm H2O in four of nine patients. The PIP was less than 75 cm H2O in only one patient and delta PDI was greater than 45 cm H2O in all ten patients evaluated. Frequency dependence of compliance was abnormal in seven of ten patients, whereas RAW was increased in only three of eight patients. The TLC and DCO were less than 80 percent of the predicted values in ten of 16 and 14 of 16 patients respectively. There was no correlation between the level of extractable nuclear antigen and pulmonary function abnormalities. The study did not identify significant abnormalities in respiratory muscle function.     

Renal involvement in mixed connective tissue disease: a longitudinal clinicopathologic study

Eleven of 30 patients with MCTD, followed for a mean of 10 years, developed immune complex nephropathy (five membranous, two mesangial, one mixed, and one sclerosing) with NS in nine of 11. Another patient had membranous nephropathy at autopsy. Patients with renal disease tended to have more systemic manifestations than those without. NS was at times of abrupt onset, recurrent, and/or persistent. Anti-RNP and serum complement were not helpful in predicting nephritis. Seventy-two percent of nephropathy and 62% of NS episodes resolved or improved after corticosteroid therapy. Five patients became hypertensive, two developed chronic renal failure and required chronic dialysis, and one needed acute dialysis twice. One patient progressed to focal proliferative crescentic nephritis with necrotizing arteritis. Three patients with nephropathy died, two of pulmonary hypertension with acute cor pulmonale and one of overwhelming sepsis. Nephropathy is relatively common in MCTD, is associated with substantial morbidity, and with the risk of hypertension and chronic renal failure.     

Vasculitis in the connective tissue diseases

Vasculitides in the setting of connective tissue diseases are generally thought to be infrequent and relatively little is written about them. They are, however, important both because they may pose diagnostic and therapeutic challenges and affect prognosis. In each of the connective tissue diseases, vasculitis can present in various clinical and pathologic forms adding to their diagnostic and therapeutic difficulties. This article reviews recent information on the frequency, characteristics, and possible pathogenic mechanisms of the vasculitides occurring in patients with the main connective tissue diseases.     

Liver abnormalities in connective tissue diseases

The liver is a lymphoid organ involved in the immune response and in the maintenance of tolerance to self molecules, but it is also a target of autoimmune reactions, as observed in primary liver autoimmune diseases (AILD) such as autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis. Further, the liver is frequently involved in connective tissue diseases (CTD), most commonly in the form of liver function test biochemical changes with predominant cholestatic or hepatocellular patterns. CTD commonly affecting the liver include systemic lupus erythematosus, antiphospholypid syndrome, primary Sjögren's syndrome, systemic sclerosis, dermatomyositis, polimyositis, and anti-synthetase syndrome, while overlap syndromes between AILD and CTD may also be diagnosed. Although liver cirrhosis and failure are extremely rare in patients with CTD, unusual liver conditions such as nodular regenerative hyperplasia or Budd–Chiari syndrome have been reported with increasing frequency in patients with CTD. Acute or progressing liver involvement is generally related to viral hepatitis reactivation or to a concomitant AILD, so it appears to be fundamental to screen patients for HBV and HCV infection, in order to provide the ideal therapeutic regimen and avoid life-threatening reactivations. Finally, it is important to remember that the main cause of biochemical liver abnormalities in patients with CTD is a drug-induced alteration or coexisting viral hepatitis. The present article will provide a general overview of the liver involvement in CTD to allow rheumatologists to discriminate the most common clinical scenarios.     

Human immunodeficiency virus-related connective tissue diseases: a Zimbabwean perspective

Our clinical experience with patients in Zimbabwe suggests that an arthropathy may be a feature of HIV disease. This takes two forms: the first is a reactive arthropathy usually affecting the large, lower limb joints with no other clinical features of a connective tissue disease. The second form is seen in association with features of complete or incomplete Reiter's syndrome with involvement of large and small peripheral joints (having an asymmetric distribution). Although this arthropathy may been seen in association with HIV positive asymptomatic disease and often is the reason for first presentation at hospital, the majority of our patients have clinical features of persistent generalized lymphadenopathy, plus or minus features of constitutional illness (such as fever, weight loss, and diarrhea). A small percentage of our patients have arthropathy in association with secondary systemic infection. Other locomotor conditions have been observed, although their numbers are too small to determine whether or not they truly are related to HIV disease. In Zimbabwe there is no association between the development of HIV-associated arthropathy and the presence of HLA-B27.     

Methotrexate therapy in connective tissue diseases: a review of the literature

Low dose methotrexate (MTX) therapy is now widely used with considerable success in refractory rheumatoid arthritis. Experience with the drug in other connective tissue diseases and vasculitis has been limited. This article reviews hypotheses about the mode of action of MTX, pharmacology and pharmacokinetics, reasons to use cytotoxic agents in the different diseases, and results of MTX therapy. Risk factors, adverse reactions and drug interactions of MTX with other drugs are mentioned. Although based on a small number of patients, data about remarkable clinical improvement in polymyositis, systemic lupus erythematosus and vasculitis suggest that MTX is a potent and effective drug in connective tissue diseases other than rheumatoid arthritis. However, controlled trials are needed to assess the exact value of the drug in these diseases.