BACKGROUND: Giant cell arteritis (GCA) or temporal arteritis (TA) is a necrotizing medium to large vessel arteritis of unknown etiology that was described in 1932 by Horton. It commonly afflicts elderly people and may present with diverse manifestations such as severe headache, impairment of vision, polymyalgia rheumatica, tenderness of the temporal arteries, a highly elevated erythrocyte sedimentation rate (ESR) and a characteristic abnormal temporal artery biopsy. It is rare in individuals less than 50 yrs old. Age of more than 50 yrs old is the first of five clinical criteria formulated by the American College of Rheumatology. In the largest series reported, all patients were ?50 years, and 95% of them were >60 years. In the last three decades, only 13 patients <40 years old have been reported with TA. We report here a case of a relatively young patient presenting with clinical features of TA with a negative temporal artery biopsy.
SETTING: St. Luke's Medical Center- a tertiary care hospital
KEYWORDS: Giant cell arteritis; temporal arteritis
CASE REPORT: Patient is a 41 y/o male who presented with a 3 week history of severe left sided frontal headache with intermittent episode of moderate grade fever, blurring of vision, diplopia on the left eye and left jaw claudication. This was followed by an onset of moderate pain over the right shoulder with limited range of motion. Pertinent physical examination revealed a pulsating, warm and tender to touch left temporal artery, with scalp tenderness. The right temporal artery was essentially normal. There was no Brundzinksi's and Kernig's signs. Other neurologic examination were unremarkable. Laboratory investigations revealed: Complete blood count (CBC) Hgb 11.6 g/dl, Hct 33.5%, RBC 4.06 mil/mm3, white blood count at 6,240 mm3, neutrophils 68%, lymphocytes 22%,monocytes 9% and platelet count of 144,000/mm3 ,ESR 90 mm/hr., CRP 34.9 mg/dl. Brain CT scan showed normal results as well as cerebrospinal fluid analysis. A left temporal artery biopsy with length of 2cm was obtained. It was grossly palpably normal but histopathology revealed intimal fibrosis of the tunica media devoid of any inflammatory infiltrates. He was managed as a case of temporal arteritis and was started on prednisone at 1mg/kg/day with remarkable improvement and had follow-up 3 months later free of complications on tapering dose of corticosteroids.

Human ; Male ; Adult ; Adrenal Cortex Hormones ; Diplopia ; Giant Cell Arteritis ; Lymphocytes ; Neutrophils ; Polymyalgia Rheumatica ; Rheumatology ; Temporal Arteries ; Tunica Media ;

INTRODUCTION: Hematologic manifestations of Systemic Lupus Erythematosus (SLE) such as hemolytic anemia, leucopenia and thrombocytopenia are among the common causes of morbidity and hospitalization among patients. This is a case report of a patient presenting with refractory cytopenias.
CASE: The  patient  is  a  42-year-old  female,  diagnosed with  SLE,  having  met  the  Systemic  Lupus  Erythematosus  International  Collaboration  Clinics  Criteria  for  diagnosis Persistent  serositis  (pleural  effusion  and  ascites) and worsening of anemia prompted treatment with oral corticosteroid (prednisone 1.0 mg per kg per day) which showed improvement of shortness of breath and  pleural effusion. Cytopenias persisted despite increasing IV pulse steroid to 6.0  mkd  prednisone then further to 13 mkd prednisone.Rituximab given as 1.0 g infusion once every two weeks for two doses which resulted to improvement of anemia, thrombocytopenia and serositis. 
CONCLUSION: Hematologic manifestations may present as life threatening complications of lupus.Most cases are responsive  to  corticosteroid  therapy,however,in a few refractory cases,less used conventional treatment such as rituximab,may provide significant response.

Human ; Female ; Adult ; Serositis ; Prednisone ; Rituximab ; Leukopenia ; Lupus Erythematosus, Systemic ; Thrombocytopenia ; Lupus Erythematosus, Discoid ; Anemia, Hemolytic ; Pleural Effusion ; Adrenal Cortex Hormones ; Dyspnea

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.
Lupus Erythematosus, Systemic ; Lupus Nephritis ; Survival

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.

Human ; Female ; Middle Aged ; Adult ; Histiocytic Necrotizing Lymphadenitis ; Anemia, Iron-deficiency ; Lymphadenitis ; Lymphatic Diseases ; Arthritis ; Arthralgia ; Lymphadenopathy ; Methylprednisolone ; Adrenal Cortex Hormones

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.
dermatomyositis ; interstitial lung disease ; immunomodulator