Connective Tissue Disorders Present With Pulmonary Symptoms About 25% of the Time

Pulmonary manifestations were found in about a quarter of connective tissue disorder (CTD) patients, according to a paper published in the journal Cureus.

Investigators from medical centers in Pakistan conducted a cross-sectional study including 290 patients with rheumatic disorders in order to determine the prevalence and patterns of pulmonary manifestations in CTD patients at their hospital. The study authors wrote that pulmonary manifestations are prevalent among CTD patients, and most patients will show signs of involvement of the lung, vasculature, pleura, and diaphragm throughout the course of their disease. They added that these patients can have a compromised immune system and methotrexate and leflunomide may have negative effects.

Subsequently, the treatment, prognosis, and management of the disease is difficult, the study authors wrote, and most be done using a multidisciplinary approach.

A majority of the patients were female and the average age of the group was about 35 years, the study authors said; most of the patients were under 40 years of age. The most commonly observed rheumatic disorder was rheumatoid arthritis, found in 70% of the patients, though more often noted in patients older than 40 compared to those younger than 40.

The second most common rheumatologic disorder was systemic lupus erythematosus (SLE), found in 14% of the patients and most often in the younger set of patients. The study authors also recorded the patients’ treatment history and immunological test findings.

The investigators found pulmonary manifestations in 27% of the patients; the remaining 73% were free of pulmonary manifestations. Pulmonary arterial hypertension was the most commonly observed disease, the study authors said, found in 11% of the patients with pulmonary manifestations. The next most common disease was nonspecific interstitial pneumonia, found in 9% of the patients. Pleural effusion was noted in 5% of patients, usual interstitial pneumonia was present in 5%, and interstitial pneumonitis was 4%, and tuberculosis was nearly 2%.

Hypersensitivity pneumonitis and pulmonary embolism were found in 1 patient each, the study authors observed.

The authors did not find a statistically significant association between the age grounds and distributions of patterns of pulmonary manifestations. However, the presence of pulmonary manifestations was found to be linked to SLE, mixed CTD, scleroderma, overlap syndrome, and dermatomyositis, but less associated with rheumatoid arthritis.

Almost all of the patients were on steroids, and 90% were being treated with hydroxychloroquine. Another two-thirds were being treated methotrexate, the study authors wrote.

And while this study was conducted at “one of the largest public-sector hospitals of Karachi [Pakistan],” the study authors noted, the fact that it was single-center and observational in design could be a limitation of their findings.

“Pulmonary manifestations are frequently found in several CTDs at some juncture during the tenure of the disease,” the authors concluded.

“Unfortunately, a few of these manifestations may go undetected as they may not show any symptoms such as interstitial fibrosis which may gradually progress in the patient eventually resulting in a fatality. Thereby, it is essential to recognize the disease in its early stages and introduce therapy and treatment accordingly which may aid in lowering the rates of death and morbidity amongst patients.”

Reference

Dahani A, Arain S, Riaz A, Khan F, Jabeen R. Prevalence and pattern of pulmonary manifestation in patients with connective tissue disorder. Cureus 12(4):e7618. doi:10.7759/cureus.7618.