Mixed connective tissue disease in Filipinos - A 13-year retrospective review of 14 cases in the Philippine General Hospital

BACKGROUND: Mixed connective tissue disease (MCTD) is a distinct autoimmune disease with overlapping features of different connective tissue diseases and a broad spectrum of presentation and prognosis. Our aim is to present the clinical profile of a cohort of Filipinos with MCTD.METHODS: Charts with diagnosis of MCTD based on the Alarcon-Segovia Criteria from the Philippine General Hospital Adult Rheumatology clinic from January 1999 to December 2011 were reviewed for demographics, clinical presentation, and management. Descriptive statistics were applied.RESULTS: Fourteen patients were identified to have MCTD. All were females. The median age at symptom onset, and at diagnosis, was 30.5, and 31.5 years, respectively, with two having juvenile onset of symptoms. Mean interval from symptom onset to diagnosis is two years. Patients are being followed up for the past mean of 3.5 years.All eight women    who wanted children were able to conceive, and among them only two had fetal losses but were negative for antiphospholipid antibodies. Chief complaint was most commonly joint pain (67%) followed by skin tightness (13%). Systemic lupus erythematosus (SLE) was the most frequent initial diagnosis (43%). Majority had initial constitutional symptoms with generalized weakness and fatigue being the most frequent (93%). Most common physical findings in different body systems are as follows: musculoskeletal - arthritis (100%); vascular - Raynaud's phenomenon (93%); cutaneous- skin tightness (71%); gastrointestinal - dental caries (57%); hematologic - anemia of chronic disease (50%); cardiopulmonary - accentuated pulmonary component of the second heart sound and right ventricular hypertrophy (21% each); neurologic - peripheral neuropathy (21%); renal - proteinuria (21%); endocrine - autoimmune thyroiditis (21%).Half have anemia of chronic disease. Only three (21%) have proteinuria and were below nephrotic range. Twelve out of 12 have elevated sedimentation rates. For serologic studies, all have speckled ANA  and very  high  titers  of  anti-U1RNP;six of six patients have  normal  rheumatoid  factor(RF) titers; one    out    of  two  have  positive  anti-Ro;two out of four have anti-Ds DNA; none of two have anti-SCL70.Majority of the chest x-rays,electrocardiograms,echocardiographs  were  normal.Three have pulmonary hypertension. One out of four has restrictive lung disease on pulmonary function test. Most are in remission and are on low-dose prednisone (79%), hydroxychloroquine (50%), nifedipine (36%) and methotrexate (21%).CONCLUSION: This is the first study that detailed clinical and laboratory features of Filipinos with MCTD diagnosed using the Alarcon-Segovia criteria. Most clinical features, disease activity, and management are concordant with international data. Possible peculiarities include fewer interstitial lung disease, esophageal dysmotility, thrombocytopenia and leucopenia, and RF titers. We intend to add newly diagnosed patients and pursue this cohort for us to better understand the course of MCTD in Filipinos that could translate to better patient care.

Correlation between disease activity, functional capacity, and health-related quality of life of Filipinos with ankylosing spondylitis

OBJECTIVE: The study aimed to evaluate the relationships between disease activity, functional capacity,  and  health-related  quality  of  life   (HRQoL) in   Filipino   patients   with   ankylosing   spondylitis (AS).METHODS: Filipino AS patients gathered from the Rheumatology Clinic of the Philippine General Hospital (PGH) and from selected tertiary hospitals  in Metro Manila who have been  diagnosed  via  the  Revised New  York  Criteria  were  included.  Disease   activity  was measured using the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), while functional capacity and HRQoL were measured using the Bath Ankylosing Spondylitis Functional  Index  (BASFI)  and Short Form (SF)-36, respectively.  Pearson's  correlation  was used to analyze  the  relationship  between  BASDAI and   BASFI,   and   between   BASDAI   and  SF-36.RESULTS: Twenty-four patients entered the study.  Mean age was 38.75 ± 12.7  years  and  mean  age  at  diagnosis  was  31 ±  12.29  years.   Symptoms  occurred for  a   mean  of   9.0  ±   6.97  years  with  mean      duration of symptom onset to  diagnosis  of  3.38  ±  5.14  years.  Study subjects had mild to moderate disease activity. Functional  capacity  was most   impaired  in   performing a full day's activities at home or at work.  HRQoL  was highest in vitality and lowest in emotional role,  with  mental health components generally showing higher scores than physical health components. Pearson's correlation showed moderate positive correlation between BASDAI and BASFI (r=0.6016, p=0.0012) and moderate negative correlation between BASDAI  and  the  physical  health  domain  (r=  -0.6916,  p=0.0001)  and mental health domain (r= -0.3575, p=0.0863)  of SF-36. However, only the first two correlations were statistically  significant.CONCLUSION: Filipino AS patients with higher disease activity have more functional disability and poorer physical  HRQoL.

A three-month follow up of musculoskeletal manifestions in chikungunya fever

BACKGROUND: Chikungunya virus (CHIKV) is a mosquito- borne  alphavirus  of  the  family  Togaviridae  transmitted  to humans by the Aedes spp. mosquitoes, causing Chikungunya   Fever  (CHIKF).OBJECTIVE: This study aims to describe the course and outcome of musculoskeletal (MSK) manifestations in patients  with  CHIKF  seen  over  a   three-month     period.DESIGN: This is a prospective descriptive  study.  Seventy  patients  with  fever,  rash,  and  arthritis  seen   at the University of the Philippines-Philippine General Hospital  and  private  arthritis  clinics  were  collected from August - December 2012.  Demographics  and course   of   arthritis   were  described.RESULTS:  Seventy   patients,   53   (68.6%)   female,   with  a mean age  of  39.2  ±13.50  were  diagnosed  with CHIKF. All cases were from the Metropolitan  Manila area. Of these, 15 (21.4%) had  family  members  affected. Twenty-four patients (34.3%) were either employed or students. The most common presenting symptoms were fever (94.3%), arthritis or athralgia  (98.6%), and rash (87.1%).  The  most  common  joint  areas involved were the ankles  (60.0%),  the  wrists (40.0%) and the small joints of the hand (51.4%). Twenty-seven (47.3%) had symmetric  arthritis.  Thirty- seven   cases   (52.9%)   had   arthralgia   or   arthritis    for at least  six  weeks.  By  the  end  of  the  follow-up  period, only four (5.7%) had persistent musculoskeletal symptoms. Age and  sex  were not  found to  be  factors in  determining  chronicity  of  arthritic  symptoms  (p   =   0.104   and   p=0.58  respectively). Of the seventy patients, 31 (44. 3%) were confirmed  cases  of  CHIKF--  29 had  (+)   CHIKV  IgM   by ELISA,  and  two   had   (+)   CHIKV   PCR.   Twenty-  one (67.7%) had  persistent  arthritis  of  at least  six  weeks. Treatment  consisted  of continuous  NSAIDs  for  at  least  two   weeks   and   some   received   steroids.  In this subgroup, age and sex were not shown to  correlate with chronic arthritis (p=0.47 and p=0.05 respectively).CONCLUSION: This report on a recent outbreak of CHIKF showed the classic triad of fever, rash,  and  arthritis.  There  was  no  correlation  between  age  and  chronicity of  arthritic  symptoms.  Sex,  likewise,  did  not  appear   to influence chronicity. The disease occurred in family clusters. Patients required continuous treatment with NSAIDs and  some  had  to  be given  steroids.  Most cases   resolved   by   12 weeks.

Second-line treatment of Filipino patients with rheumatoid arthritis included in the rheumatoid arthritis database and registry (RADAR) of the Philippine General Hospital from 1996-2010 A 2011 preliminary report

INTRODUCTION: Treatment  of  rheumatoid  arthritis  (RA) has evolved over the past  20  years, aiming at  remission with the use of disease modifying drugs (DMARDs) and recently with biologic agents. Treatment  of  Filipino  patients  with   RA   has   so   far, not  been  described  in literature.   The   University   of the Philippines-Philippine General Hospital (UP-PGH) Rheumatology Section established the rheumatoid arthritis database and registry (RADAR) to better understand   RA   as   it   affects   the   Filipino patient.OBJECTIVE: To  describe   the   treatment   received   by a cohort of Filipino  patients  with  RA  seen  in the UP-PGH and entered in the RADAR over three time  periods   from   1996   to  2000.MATERIALS AND METHODS:This   is   a   review   of   data  on treatment of all cases entered into the RADAR diagnosed   with   rheumatoid   arthritis    by    the  1987  ACR  Criteria,  between  1996   to   2010.   Cases were grouped into three five-year periods: 1996-2000 (Period  1),   2001-2005  (Period  2),   and   2006-2010  (Period3)based on initial prescription of the  drugs.  DMARDs  given were listed and trend in the rate of use was extracted. These were  then  compared  across  the three time periods.  Descriptive  statistics  using  ratios and   proportions   was   employed   on   the  data.RESULTS: Two-hundred-twenty-four (224) cases were entered  into  the  RADAR  at the  time  of  this  study   and  all  received  DMARDs.  Methotrexate (MTX)  was  the  most  common  DMARD  used  (89%).   Of   these,   75% were on MTX  monotherapy.  Hydroxychloroquine was given in 25%, with 11% on HCQ monotherapy. Combination  treatment  with  MTX  and  HCQ  was  used  in 14%  of  cases.  There  was  an   increasing   rate   of MTX use across Periods 1-3 (87%, 89% and 90%, respectively).  On the  other  hand,  a  decreasing  rate   in the  use  of  HCQ  was   noted   (43%   in   Period   1,  24%   in   Period   2,   and   21%   in   Period   3).   Only   9% of patients received biologic  agents  in combination with  MTX.CONCLUSION: This  study   reports  the   use   of   DMARDS  in a cohort of patients entered in the UP-PGH RADAR. Methotrexate monotherapy was the most common treatment used, and this increase  continued  over  the  three  observation  time   periods.   Hydroxychloroquin was used in one fourth of  patients,  and  its  use declined over the three time periods. Biologic DMARDS was rarely used, and when given, it was combined   with  MTX.

A Filipino with polyangiitis overlap syndrome and associated multiple infections

BACKGROUND: Polyangiitis overlap syndrome (POS),a systemic vasculitis not classifiable into well-defined syndromes, is  diagnosed based on combined characteristics of two or more primary systemic vasculitides, such as Takayasu arteritis  and anti-neutrophil cytoplasmic antibody (ANCA)-associated  vasculitis  (AAV), but not two AAVs. Our objective is to   present such,   with associated  multiple  infections.CASE: A  25-year-old  Filipino  female  had  six  years  of  recurrent  purpura,  debilitating  joint  pains,  hemoptysis,  rhinorrhea, epistaxis, eye redness, dyspnea,  and  abdominal  cramps. She was cachectic, had oral ulcers, bibasal  crackles,  polyarthritis,  and  generalized  purpura.   Work-up   showed   anemia,   eosinophilia  excluding   parasitism,   elevated inflammatory markers, and  positive cytoplasmic (c)-ANCA and anti-proteinase 3. There   was   chronic sinusitis on rigid rhinoscopy, middle to lower lung field  interstitial   infiltrates   on   radiographs, pulmonary vasculitis   on  chest   CT,   moderate pulmonary hypertension on  echocardiography,  but  normal  spirometry.  Skin  biopsy  showed  leukocytoclastic vasculitis without  necrotizing  granulomas.Serology showed chronic hepatitis B infection.   Pseudomonas  aeruginosa and  methicillin-resistant Staphylococcus  aureus  grew  from  bronchial  washings and   nasal   swabs,   respectively. ASO titer was high. Human  immunodeficiency  virus  infection  was  ruled  out.  She   was   diagnosed with POS,with features of two AAVs- limited granulomatosis   with   polyangiitis   (GPA)   and   Churg-Strauss   syndrome  (CSS), associated with   multiple  infections,   and  was  given naproxen and culture-guided   antibiotics  (ciprofloxacin, clindamycin, penicillin),with partial resolution of symptoms.  Prednisone   10  mg once  a  day (OD)  was   started  two weeks   after hepatitis B reactivation prophylaxis   with   lamivudine 100mg OD. Complete   resolution of skin lesions and upper airway symptoms and    inflammatory  marker  improvement  were observed after 12  weeks. She is  maintained on prednisone   5.0   mg   daily.CONCLUSION AND RECOMMENDATIONS: This   is   the   first   reported   case   of   POS   involving   two   AAVs   -   limited   GPA   and   incomplete   CSS,   associated   with   multiple   pathogens.Identifying  POS  is  important;    inability to  classify patients  into well-recognized  vasculitic  syndromes  delays  treatment.  The  infections likely  perpetuated   the   vasculitis,  and  both antimicrobials  and  immunosuppression were  necessary  to  induce   disease   remission