1.A case of Churg-Strauss syndrome presenting with foot drop.
Limgenco-Hipe Juneth Ria R. ; Manapat-Reyes Bernadette Heizel
Philippine Journal of Internal Medicine 2015;53(2):1-5
BACKGROUND: Churg-Strauss syndrome (CSS), or eosinophilic granulomatosis with polyangiitis, is a rare syndrome that affects small- to medium-sized arteries and veins. Criteria for the diagnosis include: asthma (wheezing, expiratory rhonchi), eosinophilia of more than 10% in peripheral blood, paranasal sinusitis, pulmonary infiltrates (may be transient), histological proof of vasculitis with extravascular eosinophils, and mononeuritis multiplex or polyneuropathy. The worldwide incidence of CSS is approximately 2.5 cases per 100,000 adults per year and its incidence in the United States is one to three cases per 100,000 adults per year.1 In the Philippines, the exact incidence is unknown with very few published case reports about it.
SETTING: University of the Philippines-Philippine General Hospital (UP-PGH), a tertiary training hospital in Manila, Philippines
THE CASE: A 40-year-old Filipino male with a history of adult onset asthma and recurrent sinusitis manifesting with inability to dorsiflex the left ankle (foot drop), various dermatologic lesions, and arthralgia. Complete blood count showed hypereosinophilia. Electromyography revealed asymmetric moderate to severe sensory and motor denervation of limbs compatible with polyneuropathy. Skin biopsy revealed lymphocytic vasculitis. P-ANCA was positive. During his incumbent hospitalization, the skin lesions, arthralgia and neurologic manifestations improved on administration of high dose steroids. Pregabalin was used to control pain secondary to the mononeuritis multiplex.
SIGNIFICANCE: To report a rare case of ChurgStrauss syndrome presenting as foot drop. This case highlights the importance of considering ChurgStrauss syndrome among adult patients presenting with neurologic complaint (inability to dorsiflex the left ankle/foot drop) and various dermatologic lesions.
Human ; Male ; Adult ; Churg-strauss Syndrome ; Respiratory Sounds ; Mephenoxalone ; Antibodies, Antineutrophil Cytoplasmic ; Pregabalin ; Asthma ; Granulomatosis With Polyangiitis ; Pulmonary Eosinophilia ; Mononeuropathies ; Polyneuropathies
2.Cardiac tamponade as a rare manifestation of systemic lupus erythematosus: A report on four cases in the Philippine General Hospital.
Aherrera Jaime Alfonso M. ; Manapat-Reyes Bernadette Heizel D. ; Lantion-Ang Frances Lina ; Manguba Alexander ; Salido Evelyn O. ; Punzalan Felix Eduardo ; Corpuz Allan D. ; Magallanes Jonray
Philippine Journal of Internal Medicine 2015;53(2):1-8
SYNOPSIS: Cardiac tamponade among systemic lupus erythematosus (SLE) patients is an unusual event. The pericardial effusion may be a consequence of uremia, infections in the pericardium, or the lupus pericarditis itself. We present four atypical cases of cardiac tamponade from pericarditis of connective tissue disease (CTD), all of which were treated with drainage and immunosuppressants. Due to the rarity of this combination, management was a challenge.
CLINICAL PRESENTATION: Four females each sought consult for dyspnea associated with typical manifestations of connective tissue disease such as arthritis, characteristic rashes, serositis, typical laboratory features, and a positive ANA and/or anti-dsDNA. The first three cases fulfilled the criteria for SLE, while the fourth fulfilled the criteria for SLE-dermatomyositis overlap syndrome. Echocardiography was done due to suspicion of pericardial involvement and revealed massive pericardial effusion in tamponade physiology in all cases.
DIAGNOSIS: Cardiac tamponade from serositis due to connective tissue disease [SLE (case 1 to 3) or SLE-dermatomyositis overlap (case 4). Other common etiologies of tamponade such as bacterial, tuberculous, malignant, and uremic pericardial effusion were ruled out by clinical and laboratory tools, including Gram stain and culture, cytology, PCR, and biochemical testing. The pericardial fluid of the first case tested positive for lupus erythematosus (LE) cells, indicative of lupus serositis.
TREATMENT AND OUTCOME: All patients underwent pericardial drainage via tube pericardiostomy. They received high dose glucocorticoids after infectious etiologies for the pericardial effusion were ruled out. The fourth case with the overlap syndrome, however, required more immunosuppressants using azathioprine and methotrexate. Resolution of pericardial effusion was noted with this approach. Three of four were discharged improved, however, the third case suffered from worsening nephritis and pulmonary hemorrhage leading to her demise.
SIGNIFICANCE AND RECOMMENDATIONS: Four cases of cardiac tamponade as a manifestation of connective tissue disease were presented. Literature underlines the rarity of this condition anytime during the course of SLE. Despite this, SLE should be considered as one of the differential diagnosis of cardiac tamponade, especially in patients who manifest with multi-systemic findings. Likewise, massive pericardial effusion should be considered in patients with a connective tissue disease presenting with subtle evidence of pericardial involvement. It requires timely identification and treatment with high dose steroids, after other causes such as infections have been excluded. Immediate drainage through pericardiocentesis or pericardiostomy in combination with immunosuppressants may be life-saving.
Human ; Female ; Adult ; Adolescent ; Pericardiocentesis ; Pericardial Effusion ; Azathioprine ; Cardiac Tamponade ; Methotrexate ; Glucocorticoids ; Serositis ; Dermatomyositis ; Immunosuppressive Agents ; Pericardial Fluid ; Neutrophils ; Lupus Erythematosus, Systemic ;
3.Clinical profile of patients with tuberculous arthritis admitted in a tertiary hospital in the Philippines
PhilippinesJonnel B. Poblete ; Bernadette Heizel D. Manapat-Reyes ; Maria Sonia S. Salamat
Acta Medica Philippina 2022;56(2):21-25
Background:
Tuberculous arthritis is a rare extra-pulmonary manifestation of tuberculosis (TB) that can lead to significant disability when left untreated. This study described the clinical profile of patients with TB arthritis admitted in a tertiary hospital in the Philippines.
Methods:
We reviewed medical records of 65 patients with TB arthritis admitted in the Philippine General Hospital from 2006 to 2019.
Results:
Majority of patients were male (41, 63.0%) and the mean age upon diagnosis was 46.7 ± 1.7 years (20– 85). Majority (33, 50.8%) had underlying co-morbidities including hypertension (16, 24.6%), type 2 diabetes mellitus (10, 15.4%), and chronic kidney disease (8, 12.3%). A history of previous TB treatment (10, 15.4%), intake of immunosuppressive drugs (7, 10.8%), and trauma on affected joints (13, 20.0%) were also reported. Majority presented with chronic mono-arthritis (50, 76.9%) involving the knees (27, 41.5%) and hips (21, 32.3%) with a mean duration of symptoms of 20.9 ± 3.9 months. The most frequent initial diagnosis upon admission was septic arthritis (21, 32.3%). Confirmed septic arthritis (10, 15.6%), pulmonary TB (9, 14.3%), and disseminated TB (4, 6.3%) were also observed. Majority were diagnosed based on positive acid-fast bacilli (AFB) smear, polymerase chain reaction (PCR) for Mycobacterium tuberculosis, or synovium biopsy (34, 54.0%). Anemia (43, 68.3%) and elevated inflammatory biomarkers (erythrocyte sedimentation rate, ESR [36, 97.3%] and C-reactive protein, CRP [40, 87%]) were also seen in majority of patients. The most common radiographic findings on x-ray were joint space narrowing (42, 84.0%), soft tissue swelling (22, 44.0%), and erosions (21, 42.0%). The mean length of hospital stay was 23.8 ± 2.8 days (2–113). Majority underwent surgical interventions (35, 55.6%) including debridement (28, 80.0%) and arthrotomy (25, 71.4%). All received anti-tuberculosis treatment upon diagnosis.
Conclusion
Majority of patients with TB arthritis in this study were male, with mean age in the 5th decade of life, presenting with mono-arthritis, anemia, elevated inflammatory markers, and radiologic findings of joint narrowing. All patients received medical treatment while more than half underwent surgery. There should be a high index of suspicion for TB arthritis in a patient from an endemic country presenting with chronic mono arthritis in weight-bearing joints to prevent delay in diagnosis and significant joint destruction and disability
Arthritis
;
Tuberculosis
4.Functional disability of Filipino patients with hand Osteoarthritis seen at a Rheumatology outpatient clinic of a Tertiary Government Hospital
Bernadette Heizel Manapat-Reyes ; Ivy Elline S. Afos ; Ester G. Penserga
Philippine Journal of Internal Medicine 2019;57(1):29-33
Introduction:
Osteoarthritis (OA) is a common joint disease which may cause functional limitation in daily activities. The aim of this study is to determine disability of patients with hand osteoarthritis (HOA) using the Disability of Arm, Shoulder and Hand (DASH) questionnaire and to determine association of risk factors to the development of HOA.
Methods:
This is a cross-sectional study done in a tertiary government hospital. Patients seen at the rheumatology outpatient clinic aged 40 years and above, diagnosed with primary OA of hands, knees, hips and spine based on American College of Rheumatology criteria and who gave written informed consent, were included. Patient characteristics were extracted. Descriptive statistics were used. Those with HOA were compared to those without HOA, as to biomechanical stress, menopause, body mass index (BMI) and family history. They were made to complete the DASH questionnaire. Data was analyzed using chi-square and logistics regression analysis to determine association of risk factors for HOA.
Results:
One hundred fifty patients with primary OA were included; 61.3% were between 60-79 years, 92% were female. Forty-one percent were obese, 55.3% had family history of OA. There were 88 cases of HOA (59%). Mean age at onset is 58.2±8.8 years, mean age at diagnosis is 61.8±9.6 years. The most common complaints were stiffness and pain (60.3%), stiffness only (29.5%) and pain only (10.2%). The average DASH is 28.7±15.6 (mild disability), seen in 59.1%, moderate disability in 37.5% and severe in 3.4%. Among the risk factors for the development of HOA, there was a trend for menopause, BMI and biomechanical stress to increase risk of HOA. Among the biomechanical factors, cooking, laundry and cleaning showed a trend toward increasing risk two-fold.
Conclusion
This study showed that half of patients with primary OA in the study had HOA that was mildly disabling in a majority, and moderately disabling in 1/3 of cases. There may be correlation with biomechanical factors in its development. Other important risk factors for its development need to be further studied in a larger cohort of patients.
Risk Factors
5.Chronic Tophaceous Gout in a patient with Cyanotic Congenital Heart Disease
Annalyn Q. Urbano ; Givenchy Maree D. Garcia ; Bernadette Heizel Manapat-Reyes
Philippine Journal of Internal Medicine 2018;56(1):34-37
Introduction:
Gout is a clinical syndrome resulting from deposition of urate crystals in joints causing inflammation, intense pain and even disability. In 2003 National Nutrition and Health Survey (NNHeS), the overall prevalence of gout was 1.6% and the prevalence of hyperuricemia was 28.4% of the 4498 adults >20 years old. Gout complicating cyanotic congenital heart disease (CCHD) was infrequently recognized in early reports with Somerville referring only nine patients over a period of five years in the registry of two hospitals. Recent studies have shown increased recognition, with Yamamura identifying 16% of the 92 patients with CCHD to have gouty arthritis. There is no local data on the frequency of gout among patients with CCHD
Case Presentation:
26-year-old male previously diagnosed with Transposition of Great Arteries presented with a mass on the 5th digit of left hand with pathologic bone destruction on radiograph underwent disarticulation of the same finger. Intraoperative findings revealed chalky substances that led to the referral to rheumatology section. A diagnosis of chronic tophaceous gout was considered after review of history. He had a three year history of intermittent polyarticular arthritis involving the elbows, knees and ankle joints and later developed tophi in the helices of the ears and the DIP joint of the fifth digit of the left hand. There is increasing level of hyperuricemia as the severity of arthritis progressed. He had a polyarticular gout flare post operatively. The gout attack resolved after treatment with colchicine. Allopurinol was initiated after control of gout flare with resulting decrease in uric acid level.
Discussion:
This case described a young male patient with transposition of great vessels with acute intermittent polyarthritis and tophi formation. The occurrence of gout and the complexity of the congenital heart disease have caused a considerable financial, functional and emotional instability on the patient, his parents and extended family. In patients with cyanotic congenital heart disease, the prevalence of hyperuricemia ranges from 16 to 43%. Several factors identified to be associated with hyperuricemia in CCHD in our case which include hypoxemia, polycythemia and impaired renal function. Nonetheless, management of gout in patient with CCHD is similar to the primary and secondary gout.
Conclusion
Gouty arthritis should be a differential in a patient with a cyanotic congenital heart disease complaining of joint swelling and tenderness. Early and prompt diagnosis will relieve symptoms and prevent complications that may lead to physical inactivity of patients.
Gout
;
Heart Defects, Congenital
;
Arthritis
6.Assessment of cognitive impairment in Systemic Lupus Erythematosus using the Mini-Mental Status Exam and the Montreal Cognitive Assessment Test-Filipino Version
Allan D. Corpuz ; Angeline Therese D. Magbitang ; Ana Teresa S. Hernandez ; Kenneth D. Tee ; Evelyn Osio-Salido ; Bernadette Heizel Manapat-Reyes
Philippine Journal of Internal Medicine 2018;56(2):82-88
Introduction:
Cognitive impairment (CI) in patients with systemic lupus erythematosus (SLE) presents with or without overt signs of central nervous involvement. The prevalence of CI is variable, ranging from 19-80%. It is often overlooked, leading to high healthcare costs and productivity loss. The usual tools for detection are expensive, time-consuming and not locally available. Detection of CI using the Mini Mental State Examination (MMSE) and Montreal Cognitive Assessment Test (MoCA) is more clinically relevant and practical. The objectives of this study are to determine the prevalence of CI in SLE patients using MMSE/MoCA, to determine the degree of impairment in the different cognitive domains, and to characterize patients with CI in terms of disease activity, education, and employment.
Methods:
This is a cross-sectional study of 62 SLE patients, 19 years or older, at a rheumatology clinic. Demographic and disease characteristics were collected. The validated Filipino versions of the MMSE/MoCA test were administered. Descriptive and non-parametric statistics were applied.
Results:
Most patients are female (96.77%), below collegiate level of education (58.06%), and unemployed (70.97%). Mean disease duration is 8.92 (SD±7.03) years. Mean age at diagnosis is 28 (SD±10.30) years. Hypertension is the most common co-morbidity. Most have low lupus disease activity or are in remission (80.65%). Most are on prednisone (72.58%), with an average dose of 11.88mg/day (SD±10.66). The prevalence of CI is 38.71% (MMSE-P) and 77.42% (MoCA-P). The presence of CI is not related to educational level, employment, and disease activity.
Conclusion
Cognitive impairment (CI) is common in this cohort of SLE patients. Disease activity, level of education and employment do not seem to affect its occurrence. The MMSE-P and MoCA-P are rapid tools to assess the presence of CI and should be used in clinical practice to improve the quality of care for patients with lupus.
Lupus Erythematosus, Systemic
;
Cognitive Dysfunction
;
Mental Status and Dementia Tests
;
Philippines