THE CHRONOLOGY OF PULMONARY AND CUTANEOUS MANIFESTATIONS IN SYSTEMIC SCLEROSIS RELATED INTERSTITIAL LUNG DISEASE PATIENTS: A CASE SERIES

Background: The auto-immune connective tissue diseases (AICTD) are polygenic clinical disorders having heterogeneous overlapping clinical features. Certain features like autoimmunity, vascular abnormalities, arthritis/arthralgia and cutaneous manifestations are common to them. Lung involvement can present in AICTDs in form of: pleurisy, acute/ chronic pneumonitis, pulmonary artery hypertension (PAH), shrinking lung syndrome, diffuse alveolar damage, pulmonary embolism (PE), bronchiolitis obliterans organizing pneumonia, pulmonary infections, cardiogenic pulmonary edema, etc. High-resolution computed tomography (HRCT) plays an important role in identifying patients with respiratory involvement. Pulmonary function tests are a sensitive tool detecting interstitial lung disease.Methods: The present study is an observational study carried out on 170 patients of AICTD in department of Dermatology, Venereology and Leprosy at a tertiary care centre during a period of 2 years from October 2017 to August 2019. Detailed history, examination and relevant investigations like chest X-ray, pulmonary function test (PFT), HRCT thorax were done as indicated.Results: The overall incidence of respiratory involvement was 56.7% with maximum involvement in systemic sclerosis cases (82.8% of cases). 45.7% of patients of systemic lupus erythematosus had respiratory involvement, most common being pleural effusion in 11.5%. Impaired PFT’s were seen in 82.8% cases of systemic sclerosis (SSc) and all cases of UCTD. Interstitial lung disease was seen in 34.7% and 25% cases of SSc and DM respectively. PAH was found in 15.2% cases of SSc and 9.8% cases of mixed connective tissue diseases.Conclusions: AICTD are multisystem disorders in which pulmonary involvement can be an important cause of morbidity to the patient and early detection is necessary for prevention of long-term respiratory complications.

Download Full-text

Anti-SAE1 Antibodies and Dermatomyositis: A Case Series of Three Patients

SKIN The Journal of Cutaneous Medicine ◽

10.25251/skin.4.6.15 ◽

2020 ◽

Vol 4 (6) ◽

pp. 578-584

Author(s):

Claire Wilson ◽

Brandon Roman ◽

Kurt Ashack ◽

Iris K Aronson

Keyword(s):

Interstitial Lung Disease ◽

Cohort Studies ◽

Lung Disease ◽

Patient Population ◽

Case Series ◽

Response To Treatment ◽

Laboratory Findings ◽

Cutaneous Manifestations ◽

Associated Symptoms ◽

Life Threatening

To date, few patients with anti-SAE1 dermatomyositis (DM) have been described, and usually in large descriptive cohort studies including all DM subtypes. Thus, it is increasingly important to further describe this rare subtype to solidify cutaneous findings, associated symptoms, and potential therapeutic options. Herein, this case series describes three patients with anti-SAE1 DM with respect to their clinical and laboratory findings, and also their response to treatment. All three patients eventually presented with classic cutaneous manifestations of dermatomyositis, however, Case 2 presented initially with cutaneous manifestations not described in the literature. Furthermore, two patients presented with dysphagia, with Case 2 experiencing a life-threatening dysphagia not typically described in the literature. Two of three patients also presented with early signs of interstitial lung disease. Lastly, cutaneous disease was only responsive to immunosuppressants in one patient. Unfortunately, this observational case-series consisted of only three patients, limiting the generalizability of our results. However, this study provides further support of the recalcitrant nature of this DM subtype, and commonly affected extracutaneous systems including the gastrointestinal and pulmonary systems. Larger studies of this patient population are needed to further confirm our associated findings.

Download Full-text