Progressive interstitial lung disease in a clinically quiescent dermatomyositis

@#A 60-year-old Filipino woman diagnosed with dermatomyositis was initially on prednisone and methotrexate. She eventually developed interstitial lung disease (ILD) and so methotrexate was shifted to azathioprine; however, azathioprine was discontinued due to cutaneous tuberculosis. Over eight years, the dermatomyositis was controlled by prednisone alone but the ILD worsened. This case demonstrated that the course of ILD may be independent of dermatomyositis.

Ten-Year survival analysis of Filipino patients with Systemic Lupus Erythematosus at the National Kidney and Transplant Institute

Introduction@#Systemic lupus erythematosus (SLE) is increasingly being diagnosed in our country. This study aims to describe the clinical features, management strategies and outcome of patients with SLE during a ten-year period.@*Methods@#This is a retrospective cohort study of patients first diagnosed with SLE at the National Kidney and Transplant Institute in 2004 who were then followed up in the next ten years.@*Results@#Eighty-five patients were first diagnosed with SLE in 2004. The mean age was 28.1±12.03 years old. Hypertension (34.12%) was the most common co-morbid illness. Renal involvement (74.12%) was seen in a majority but only those with cardiopulmonary manifestations (mean=0.71 years, p=0.030) significantly affected survival. Eleven patients (12.94%) expired during the study period. Active disease and infection were the most common causes of death. Biopsyproven lupus nephritis had a significantly higher survival rate (mean=10.57 years, p=0.006). Those on hemodialysis had a significantly lower survival time (mean=8.82 years, p=0.040). Discussion: The estimated 10-year cumulative survival rate of patients with SLE in our cohort was 75%. This is comparable to the rates reported in some countries. Regular follow-up at six to eight weeks intervals with more frequent follow-up for patients with an SLE flare and/or on intensive immunosuppression was the most likely reason for studies reporting higher survival rates. The disparity in the survival rates may also be attributed to the frequency of exacerbations with better survival among those who never had exacerbations. The most common cause of death was due to septic shock secondary to pneumonia. The authors believe that one factor that was contributory to death was the degree of immunosuppression as observed in studies describing high doses of corticosteroids on those who have died.@*Conclusion@#The cumulative survival rate decreased from 90% at the time of diagnosis to 75% on the tenth year which was comparable to several countries. Patients with cardiopulmonary manifestations were found to significantly affect survival in this study. Although renal involvement was the most common initial manifestation, it did not significantly affect survival similar to other studies. However, biopsyproven lupus nephritis cases had better survival since this allowed treatment to be streamlined based on the class of lupus nephritis. Active disease and infection were the most common causes of death.

Kikuchi Fujimoto disease: A series of three cases

@#<p style="text-align: justify;"><strong>INTRODUCTION:</strong> Kikuchi-Fujimoto disease (KFD) is a  rare self-limited disorder manifested by painful cervical lymphadenopathies commonly associated with fever and night sweats.This is a series of three female patients presenting with fever and lymphadenopathies diagnosed with KFD.<br /><strong>CASE:</strong> The first case is a 34-year-old female admitted due to fever of 10 days associated with lymphadenopathies and joint pains.Excision biopsy done showed necrotizing histiocytic lymphadenitis consistent with KFD.Other laboratories showed hypocomplementemia, positive ANA and anti-dsDNA.Patient was discharged improved with low dose oral corticosteroid and hydroxychloroquine.The second case is a 53-year-old female with fever,lymphadenopathies,polyarthritis and morning stiffness.Biopsy of the cervical lymph node was done showing KFD and lupus serologies (ANA 1:640 speckled, anti-dsDNA and anti-Smith) revealed positive results as well.Patient was then diagnosed with SLE and was started on low dose oral corticosteroid and hydroxychloroquine which resulted to resolution of fever and gradual resolution of lymph nodes on out-patient follow up.The last case is a 45-year-old female admitted due to persistent fever, painful lymphadenopathies and headache. Serological work-up including autoantibody tests for SLE were all unremarkable but showed associated iron deficiency anemia. Biopsy of the cervical lymph node showed Kikuchi's disease. Patient was discharged with oral methylprednisolone.<br /><strong>CONCLUSION:</strong> The rarity of KFD makes defining an autoimmune etiology a challenge to clinicians.Careful disease course follow up is then recommended for patients who initially lack parameters for SLE diagnosis.</p>

Kikuchi Fujimoto disease: A series of three cases

INTRODUCTION: Kikuchi-Fujimoto disease (KFD) is a  rare self-limited disorder manifested by painful cervical lymphadenopathies commonly associated with fever and night sweats.This is a series of three female patients presenting with fever and lymphadenopathies diagnosed with KFD.CASE: The first case is a 34-year-old female admitted due to fever of 10 days associated with lymphadenopathies and joint pains.Excision biopsy done showed necrotizing histiocytic lymphadenitis consistent with KFD.Other laboratories showed hypocomplementemia, positive ANA and anti-dsDNA.Patient was discharged improved with low dose oral corticosteroid and hydroxychloroquine.The second case is a 53-year-old female with fever,lymphadenopathies,polyarthritis and morning stiffness.Biopsy of the cervical lymph node was done showing KFD and lupus serologies (ANA 1:640 speckled, anti-dsDNA and anti-Smith) revealed positive results as well.Patient was then diagnosed with SLE and was started on low dose oral corticosteroid and hydroxychloroquine which resulted to resolution of fever and gradual resolution of lymph nodes on out-patient follow up.The last case is a 45-year-old female admitted due to persistent fever, painful lymphadenopathies and headache. Serological work-up including autoantibody tests for SLE were all unremarkable but showed associated iron deficiency anemia. Biopsy of the cervical lymph node showed Kikuchi's disease. Patient was discharged with oral methylprednisolone.CONCLUSION: The rarity of KFD makes defining an autoimmune etiology a challenge to clinicians.Careful disease course follow up is then recommended for patients who initially lack parameters for SLE diagnosis.