Progressive systemic sclerosis with unilateral osteolysis of the mandible: a unique case report and review

Objectives: This case report is aimed to discuss case findings of Progressive Systemic Sclerosis (PSS), an overview of the characteristics in the form of osteolysis on one side of the mandible, and a theoretical review. Case Report: A 30-year old male patient came to an oral surgeon after tooth extraction. Clinical extraoral examination revealed hyperpigmentation on the right side of the face. A radiological study showed widening periodontal space on posterior teeth, and the angles of the mandibular arch, the jaw branch and the mandibular condyle neck were dissolved in the form of bone resorption. Conclusion: Characteristics of Progressive Systemic Sclerosis (PSS) in radiographs appear in the form of expansion of the periodontal space and osteolysis of the mandibular angle, branch, and even condyle. This disease is caused by an autoimmune disease that affects the entire body, but it can manifest on one side of the body.

Clinical and psycho-socio-economic impact of COVID-19 pandemic on patients of the Indian Progressive Systemic Sclerosis Registry (IPSSR)

Objectives To determine the impact of COVID-19 pandemic on access to healthcare among the patients with Scleroderma. To analyse the economic and psychosocial impact and the infection prevention measures taken by them during the pandemic. Methods A 25 item questionnaire designed to assess the components of the objectives was tele-administered between October 2020 and January 2021 to the patients enrolled in the Indian Progressive Systemic Sclerosis Registry registry. Results Out of the 428 patients in the registry, 336 took part in the study. Scheduled outpatient visit was missed by 310 (92.3%) patients and 75 (22.3%) skipped prescription drugs. During the pandemic, 75 (22.3%) had a family member lose jobs. Financial difficulties were reported by 155 (46.1%) with 116 (34.5%) patients having to spend an additional of INR 4000 (2000 to 10000) [USD 54.9 (27.0– 137.4)] to continue treatment. Though 35 patients (10.4%) had at least one symptom suggestive of COVID-19, confirmed infection was only in 4. None of them needed hospitalisation or had adverse outcomes. Worsening of Scleroderma was seen in 133 (39.6%) individuals with 15(4.5%) requiring hospitalisation. Most (96%) of the patients were aware about infection prevention measures and 91(27.1%) had taken unproven prophylactic medications. Conclusion Individuals with Scleroderma in India have been affected during the pandemic due to closure of hospital services, lack of transport, loss of job and the additional financial burden. Healthcare providers should continue to educate patients to stay on their medications and encourage them to get vaccinated for COVID-19.

Rapidly Progressive Systemic Sclerosis–associated Interstitial Lung Disease After Intravesical Bacillus Calmette-Guérin Therapy for Early-stage Bladder Cancer

Despite recent advances in the management of systemic sclerosis–associated interstitial lung disease (SSc-ILD), it remains the most common cause of death and a significant contributor to morbidity.1,2 SSc-ILD is characterized by a wide spectrum of disease courses, with some patients having limited nonprogressive fibrosis, whereas others develop rapid and extensive fibrosis leading to respiratory failure.3

A 3-Year Observational Study of Patients with Progressive Systemic Sclerosis Treated with an Intensified B Lymphocyte Depletion Protocol: Clinical and Immunological Response

Background: B-cells have been suggested to play a role in the pathogenesis of systemic sclerosis (SSc), representing, therefore, a potential therapeutic target. Objectives: We aimed at investigating the 36-month outcomes of 20 SSc patients who underwent an intensified B-depletion therapy (IBCDT) scheme, including both Rituximab (RTX) and cyclophosphamide (CYC). Methods: Data from 20 severe patients (18 females and 2 males, mean age 66.7 ± 11.0 years) with diffuse SSc (anti-topoisomerase I antibody in 95%) patients with multiorgan involvement including interstitial lung disease (ILD) treated with an IBCDT were prospectively collected. IBCDT comprehended: RTX 375 mg/m2 administered for four weekly doses (on days 1, 8, 15, and 22), followed by two additional doses after 30 and 60 days, in addition to two administrations of 10 mg/kg of intravenous CYC plus three methylprednisolone pulses (15 mg/kg) and subsequently followed by oral prednisone rapidly tapered to low minimum dosage of 5 mg daily. In addition, 10 patients with more severe functional respiratory impairment at baseline were also treated with RTX 500 mg every 4 months during the first year and two times a year during the second and the third year. Results: After 36 months of follow-up, we recorded significant amelioration in N-terminal-pro-brain natriuretic peptide (NT-proBNP) levels (mean 385.4 ± 517 pg/mL at baseline to 279 ± 543 after 36 months). In addition, a significant radiological improvement of ILD in 20% of patients (4/20) and a radiological stabilization with no sign of progression of interstitial involvement in 13/20 (65%) were documented. A total of 3 out of 20 (15%) patients experienced a worsening of the ILD. No patient showed further decrease in functional respiratory parameters, including forced vital capacity, forced expiratory volume in one second, and mean values of diffusing capacity for carbon monoxide Moreover, no patient showed any change in the ejection fraction and pulmonary artery pressure when comparing values at baseline and after 24 and 36 months of observation. No severe infection, renal flare, RTX-related side effects were observed. No patient died. Conclusions: Our findings support that the IBCDT was well tolerated and might be a promising therapeutic option for the management of SSc, especially in those subjects with multiorgan involvement that includes ILD.

Role of HRCT in Interstitial Lung Disease with Radiographic Correlation

BACKGROUND Diffuse interstitial lung disease is on the rise in recent times. This could be attributed to number of causes / reasons. Environmental pollution is one of the important causes for the upswing. Increased exposure to inorganic dust, toxic fumes, certain drugs (gold, amiodarone, antibiotics etc.) and radiation can also cause ILD (Interstitial Lung Disease). METHODS This is a hospital based prospective observational study, conducted in the Department of Radiodiagnosis, NSCB Medical College and Hospital, Jabalpur. This study comprised of a total of 50 patients clinically diagnosed interstitial lung disease who had undergone x-ray chest and HRCT (High-Resolution Computed Tomography) Chest examination. RESULTS Incidence of interstitial lung disease was maximum in the middle age group of 51 – 60 years (38 %) with male predominance (58 %). Smoking habit was seen to be associated with 52 % of the cases suggesting association. 8 patients (16 %) had developed diffuse lung disease secondary to connective tissue disorder (rheumatoid arthritis, progressive systemic sclerosis and polymyositis). A confident diagnosis was made on the basis of chest radiograph alone in 11 patients (29 %). Among cases evaluated by HRCT Chest, 41 (82 %) cases showed specific patterns of ILD and nine cases (18 %) showed nonspecific patterns and were classified under unclassified IIP (Idiopathic Interstitial Pneumonia). Out of 41 cases, the most common ILD reported was IPF (20) followed by cases of idiopathic NSI, 8 cases of collagen vascular diseases, 2 cases suggestive of COP / BOOP, and smoking related ILDs were seen in 4 cases, and 2 cases of sarcoidosis were seen in the study. Occupational- and radiation-induced-ILD were seen in one case each. CONCLUSIONS Chest radiography is the initial imaging investigation in the evaluation of diffuse interstitial lung disease. HRCT is the imaging modality of choice to assess interstitial lung disease. It can detect lesion even when the chest radiograph is normal. HRCT can confirm the location, extent of disease, and pattern of disease. HRCT helps in the confident diagnosis of ILD as compared to chest radiography. Thus, it avoids lung biopsy in a large number of patients. HRCT is also useful in assessing the disease reversibility. Follow up of patients is better done by HRCT. KEYWORDS HRCT, Interstitial Lung Disease, Chest X-Ray, Ground Glass Opacity, Honeycombing

Upfront Combination Therapy With Rituximab and Mycophenolate Mofetil for Progressive Systemic Sclerosis

Systemic sclerosis (SSc) is a complex disease involving multiple pathophysiological pathways: autoimmunity, vasculopathy, and fibrosis, all of which are interrelated. Most of the damage consists of skin and lung fibrosis, and is accumulated within the first 2 years of disease in rapidly progressive patients with a serology of anti-SCL-70 or anti–RNA polymerase III (RNAP3)1.