Idiopathic Cd4+ Lymphocytopenia In Overlap Syndrome (Systemic Sclerosis With Dermatopolimyositis)

Background : Idiopathic CD4 T cell lymphocytopenia (ICL) is a rare syndrome with varied clinical manifestation, characterized with lymphopenia and decreased in CD4 level without HIV infection or other possible cause of immunodeficiency state. Autoimmune diseases might be a clinical manifestation of ICL. However, it is not known whether ICL triggered an autoimmune diseases, or it is a complication of said diseases. Objective : Awareness of ICL in patient with known autoimmune diseases whom admitted to the hospital for severe infection. Methods : This case report showed a 24-years old woman with prolonged fever since 4 moths ago. It was accompanied with oral ulcers, skin rash in face and trunks, and weakness of lower extremities. She was diagnosed with systemic sclerosis since 2016 and routinely came to rheumatology outpatient clinic in Hasan Sadikin Hospital but stopped coming for past 4 months since pandemic. Her current medication was only 4 mg of methylprednisolone. Results : She had high temperature (38.5 degree Celsius) and tachycardia. Physical examination revealed a single lymphadenopathy at neck. Raynaud phenomenon, calcinosis, and sclerodactyly was found in lower extremities. Dermatomyositis was diagnosed based by heliotropic skin rash. Laboratory tests showed leukopenia, absolute lymphocyte count 135.2 cell/mm3, absolute CD4 39/uL, CK level of 3296 and nonreactive anti-HIV. The patient underwent empirical antibiotic treatment, but unfortunately passed away. Conclusion : ICL is a rare case, following an infection, autoimmune diseases, or unspecified malignancy. Clinician’s awareness toward ICL could prevent fatal opportunistic infection which often happens to patients with immunodeficiency state.

Pulmonary Hypertension in Systemic Lupus Erythematosus with Raynaud’s Phenomenon: Case Report

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects multiple organs such as skin, joints, kidneys, heart, lungs, nervous system and blood. Pulmonary hypertension is a rare complication of SLE.1 Systemic lupus erythematosus associated with pulmonary hypertension was defined as an increase in the average pressure in the pulmonary artery at rest ? 25 mmHg with pulmonary capillary wedge pressure ?15 mm Hg and an increase in pulmonary vascular resistance.2 The prevalence of pulmonary hypertension in SLE approximately 0.5 to 17.5%. Predictors factors of the occurrence of pulmonary hypertension in LES is Raynaud's phenomenon, anti-U1RNP antibody, and antibody positive anticardiolipin.3 A woman aged 37 years came with a chief complaint of pain in the fingers and toes with black-colored wounds felt since 6 months before admission. From the anamnesis, physical examination, support to meet 5 of the classification criteria for systemic lupus erythematosus based on the 1997 ACR criteria which includes manifestations of arthritis, mucocutaneous, serositis, lupus antiokoagulan, and ANA IF positive. In these patients also found the typical signs and symptoms of Raynaud's phenomenon which leads to the symptoms of pain in the fingers of both hands when exposed to cold and pale to red when heated and has been confirmed from the results of arteriography. From the results of echocardiography reveal any pulmonary hypertension. This patient was treated with steroids, immunosuppressants and antiplatelet. The case was removed because of pulmonary hypertension is a complication LES rare and necessary sharpness in diagnosis. Patients with pulmonary hypertension of unknown or untreated can become a progressive right heart failure and lead to death.3

Effect of Vitamin D Supplementation in Disease Activity, Activity-Related Markers, Inflammatory Marker and Serum Calcium of Systemic Lupus Erythematosus Patiens: A Systematic Review and Meta-Analysis

Background: Effect of additional supplementation of vitamin D toward Systemic lupus erythematosus (SLE) disease activity still differ in results obtained between studies being conducted. The current meta-analysis systematically analyzed the effect of vitamin D supplementation on SLE disease activity with updated literature, also its effect toward other parameters. Material and Methods: Relevant literatures were obtained from PubMed database and Google Scholar. The obtained studies were analyzed using fixed effect model or random effect model. Results: Five eligible studies with a total of 318 participants were included. Vitamin D supplementation did not affect the total SLEDAI score in SLE patients with pooled mean difference of -0.96 (p =0.09; 95% CI: -2.06 to 0.14). Serum vitamin D level increased after administration of vitamin D with pooled mean difference of 12.67 (p =0.001; 95% CI: 5.04 to 20.29). vitamin D supplementation increase serum calcium levels, with pooled difference of 0.07 (p = 0.006; 95% CI: 0.02 to 0.12). Pooled results from two studies obtained vitamin D supplementation did not affect ESR, C3 and C4. Conclusions: Current meta-analysis obtained no significant changes in SLEDAI scores due to vitamin D supplementation. In contrast, serum vitamin D and serum calcium levels were increased.

The Relationship Between Modified Rodnan Skin Score (mRSS) With Pulmonal Hypertension and Lung Fibrosis in Systemic Sclerosis Patients

Background: Systemic sclerosis (SSc) is a connective tissue disease, which affects the skin, blood vessels, heart, lungs, kidneys, gastrointestinal tract and musculoskeletal system. The manifestations in blood vessels include pulmonary hypertension which can be seen from echocardiography, while the manifestations to the lungs in the form of pulmonary fibrosis are examined by high-resolution computed tomography (HRCT). Modified Rodnan Skin Score (mRSS) a method for measuring skin thickness in SSc patients. Objective: This study aims to determine the relationship between mRSS and pulmonary hypertension and pulmonary fibrosis in SSc patients. Methods and Materials: Research with a cross sectional approach at Dr Kariadi Hospital. There were 23 study subjects with SSc patients having their mRSS measured by a rheumatologist and an HRCT examination by a radiologist to see the percent of lung damage and echocardiographic examination by a cardiologist to measure the Tricuspid Regurgitation Velocity Maximum (TRV Max) as a marker of pulmonary hypertension. Normality test using Saphiro-Wilk. The Spearman rank correlation test was used to analyze the relationship between the mRSS score, pulmonary hypertension and pulmonary fibrosis. Results: The proportion of study subjects with a mean age of 40.39 years, women, and 4.43 years of illness. The mean mRSS score was 17.43. Examination of pulmonary hypertension from echocardiography found most of the light category 95.6%, heavy category 4.4%, while the HRCT examination found pulmonary fibrosis of 86.9%, normal 13.1%. There are significant relationship between mRSS and pulmonary fibrosis (r = 0.485, p = 0.019), and significant relationship between pulmonary hypertension (r = 0.63, p = 0.001) Conclusion: There is a significant relationship between mRSS, pulmonary fibrosis and pulmonary hypertension.

Should Patient with Autoimmune Inflammatory Rheumatic Diseases (AIIRD) be vaccinated with COVID-19 Vaccines?

Vaccination is a very important measure for the prevention of various infections worldwide including the recent COVID-19 disease. However, until now the COVID-19 vaccine with various platforms has not been clinically tested on autoimmune inflammatory rheumatic disease (AIIRD) patients, due to caution against possible side effects and unknown efficacy. Several recent studies proved that there is increased risk of SARS-CoV-2 infection in AIIRD patients and moreover, those patients also have worse COVID-19 outcomes. Thus, patients with AIIRD should be prioritized for vaccination because they have an increased burden of infections, including COVID-19. Many studies showed that inactivated/non-live vaccine is safe for AIIRD patients and do not cause disease exacerbations. We conclude that benefits of vaccination greatly outweigh the risks of infection and therefore, COVID-19 vaccines can also be administered safely in stable AIIRD patients.

Indonesian Rheumatologist Association (IRA) Recommendations for Diagnosis and Management of Peripheral Spondyloarthritis 2021

Objective. Spondyloarthritis recommendations are made to provide guidance in diagnosis, treatment, and monitoring of peripheral spondyloarthritis cases.Method. Literature searches were conducted online. The drafting team determines the levels of evidence (LOE) and grades of recommendation (GOR). After conducting a discussion, each recommendation that has been agreed upon by the drafting team is then classified based on the levels of evidence and grades of recommendation. The final step in the preparation of these recommendations is to determine the level of agreement (LOA) on each recommendation carried out by a team of panelists who have been appointed by IRA.Result. Thirty five recommendations regarding peripheral spondyloarthritis were compiled. There are 6 strong recommendation or GOR A for psoriatic arthritis and 8 recommendations for enteropathic arthritis.Conclusion. These recommendations provide directions for clinicians to diagnose and to manage peripheral spondyloarthritis.

Indonesian Rheumatology Association (IRA) Recommendations for Diagnosis and Management of Axial Spondyloarthritis 2021

Objective. Recommendations for spondyloarthritis are made to provide guidelines in diagnosis establishment, therapy, and monitoring of axial spondyloarthritis cases.Method. Literature searches were conducted online. The drafting team determines the levels of evidence (LOE) and grades of recommendation (GOR). After conducting a discussion, each recommendation that has been agreed upon by the drafting team is then classified based on the levels of evidence and grades of recommendation. The final step in the preparation of these recommendations is to determine the level of agreement (LOA) on each recommendation carried out by a team of panelists who have been appointed by IRA.Results. Twenty recommendations regarding axial spondyloarthritis were established. Strong recommendations or GOR A include: Ax-SpA diagnosis can be made according to the 2010 ASAS criteria, or the 1984 modified New York criteria specifically for the US; conventional radiographic examinations and MRI of the sacroiliac joints and vertebrae may be used as evaluators of disease activity and predictors of therapeutic response, as well as patients who do not respond to NSAIDs (within 4 weeks) can be administered a combination of NSAIDs and anti-TNF, and if it is not possible, a combination of NSAIDs and anti-IL-17A (Secukinumab, Ixekizumab).Conclusion. These recommendations provide a direction for clinicians to diagnose and manage spondyloarthritis.

Indonesian Rheumatology Association (IRA) Recommendations for Diagnosis and Management of Rheumatoid Arthritis

Introduction: Rheumatoid arthritis (RA) is an autoimmune rheumatic disease which often found in daily practice and requires certain considerations in recognizing clinical appearance also managing the disease as it often causes permanent joint damage, disability, even premature death. This recommendation is expected to become the latest reference for diagnosis and management of RA in Indonesia. Methods: The steering committee was formed by the Indonesian Rheumatology Association (IRA) to formulate key questions; conduct literature search, selection, and review; then formulate recommendation statements for diagnosis, therapy, and monitoring of RA. Furthermore, the steering committee determined the level of evidence and grades of the recommendations. After that, the level of agreement (LOA) was determined for each item by panelists including rheumatology consultants who have been appointed by IRA to represent Indonesia regions. Results: The steering committee established 30 recommendations including diagnosis, the role of laboratory and radiology tests, general treatment, the use of glucocorticoids, sDMARD, bDMARD, and tsDMARD. This recommendation also discusses guidelines on monotherapy, combination therapy, treatment strategies (treat-to-target), tapering, and continuous clinical remission. Treatment on co-morbidities and complications are also included in brief. Conclusion: IRA recommendations regarding the diagnosis and management of RA was made by considering various aspects such as the availability of drugs and supporting facilities, socioeconomic and cultural conditions in Indonesia, as well as the latest research that can be applied to Indonesian population.

A young lady with ANA negative SLE and Secondary Antip Phospholipid Syndrome

A B S T R A C TSystemic lupus erythematosus (SLE) is a chronic, inflammatory,autoimmune, multisystem connective multi system connective tissuedisease characterized by various autoantibodies to nuclear and cytoplasmicantigens and commonly affects the joints and a variety of organs due to anover activation of the body's immune system. There is wide heterogeneity inpresentation of SLE patients, including lung, central nervous system, skin,kidney, and hematologic manifestations. The presence of antinuclearantibodies (ANA) in serum is generally considered a decisive diagnostic signof SLE. However, a small subset of SLE patients who had the typical clinicalfeatures of SLE was reported to show persistently negative ANA tests. Ourreport describes a 24-yr-old female who presented with the clinicalmanifestations of SLE such as malar rash, photosensitivity, arthritis, oralulcer, and proteinuria. The serum autoantibodies were all negative exceptanti ribosomal P. She was also positive for lupus anticoagulantanti -coagulant. She was treated with oral prednisolone , hydroxychloroquinewith topical tacrolimus, and improved significantly. Three months after,repeat ANA, and anti-ds DNA showed persistent negativity, but lupus anti -coagulant remained positive. This case suggests that ANA may not berequired in the pathogenesis of SLE.

Correlation Between Mex-Sledai and Mean Platelet Volume In Systemic Lupus Erythematosus Patients

A B S T R A C TBackground. Systemic lupus erythematosus (SLE) is a chronic autoimmunedisease which has a variety of clinical phenotypes with a complex clinical course.The clinical phenotypes are highly variable which can be characterized by acuteattacks, active periods, controllable or remission. Mean platelet volume (MPV) is astraightforward accessible indicator of platelet activity and is associated withsystemic inflammation. The routine usage of MPV as markers for disease activityin SLE remains problematic. Early detection of disease progression is pivotal in themanagement of SLE for obtaining better outcomes. Objective. The study aimed toconfirm that the measurement of the disease severity in patients with SLE usingMex-SLEDAI has a negative correlation with the decrease of MPV value. Methods.This cross-sectional analytic descriptive study was conducted using secondary datafrom the medical records of patients with SLE older than 18 years of age who werenot taking antiplatelets in the Internal Medicine policlinic of Dr. Sardjito GeneralHospital in 2018. Patients with a previous history of cardiovascular andcerebrovascular events, malignancies, receiving a treatment for infections,including sepsis, chronic infections (i.e. tuberculosis, cytomegalovirus, herpessimplex, herpes zoster), HIV, hepatitis B or hepatitis C, and incomplete data ofmedical record were excluded. The correlation between SLEDAI Mex and MPVvalues was evaluated using the Spearman's correlation test. Results. Sixty-sevensubjects (65 women, 2 men) aged 34 ± 11 years were recruited in the study. Themedian duration of diagnosis was 38,2 ± 45,7 months. Arthritis, skin rash andphotosensitivity were identified in 81,1%, 53,7%, and 46,3% of patients,respectively. The Mex-SLEDAI score ranged from 0 to 16. The average of MPV valueis 9.73 ± 1.21 fL. A significant correlation between MPV and SLEDAI Mex wasobserved (p = 0.03 (p <0.05), r = -0.255). Conclusion. The more severe SLE diseaseactivity (based on the Mex-SLEDAI scoring), the more negative correlation with theMPV value.