Able Lawrence

Development and initial validation of the Indian Takayasu Clinical Activity Score (ITAS2010)

OBJECTIVES There are no valid instruments to measure disease activity in Takayasu arteritis (TA). We aim to provide a valid measure to assess clinical disease activity with or without incorporating acute phase reactants. METHODS The Indian Takayasu Clinical Activity Score (ITAS) was initially derived from disease manifestations scored in the Disease Extent Index (DEI.Tak). The ITAS was validated by a group of physicians scoring both live and paper cases for inter-rater reliability (IRR), convergence with BVAS, correlation with the Physicians Global Assessment (PGA) and ESR/CRP. It was further validated at a single centre in 177 patients for its ability to discriminate between active and inactive disease state at first visit and sensitivity to change in 132 active patients measured serially at two follow-up visits. ITAS-A also included graded scores for ESR/CRP. RESULTS The final ITAS2010 contains 44 items with 33 features arising from the cardiovascular system. Seven key items are weighted to score 2 and all others score 1 only. Inter-observer variability was highly satisfactory (IRR 0.97). The ITAS showed superior inter-rater agreement compared with the BVAS (IRR 0.9) and PGA (IRR 0.82). In the single-centre study, median ITAS scores at first visit were significantly higher in active disease (5.62 ± 3.14) compared with grumbling (3.36 ± 1.96) and inactive disease (1.27 ± 1.26, P < 0.0001). The therapy induced a significant decrease in the ITAS2010 but the higher ITAS-A scores remained elevated. CONCLUSION The ITAS2010, validated in over 300 TA patients and sensitive to change, is a useful measure of clinical disease activity for patient monitoring. Higher ITAS-A scores suggest poor control of active disease by current therapy.

Fibromyalgia is common and adversely affects pain and fatigue perception in North Indian patients with rheumatoid arthritis.

Objectives. Fibromyalgia (FM) has been shown to be common in patients with rheumatoid arthritis (RA), but studies on Asian patients are lacking. It remains unclear whether FM has an adverse influence on pain, fatigue, quality of life, and mood in these patients, and what its relationship is with disease activity. We studied prevalence and effects of FM in North Indian patients with RA and associations of RA with disease activity. Methods. This cross-sectional study included 200 RA patients and an equal number of controls. Presence of FM was defined using the American College of Rheumatology 1990 criteria. Pain and fatigue scores were assessed using a 10 cm visual analog scale. Quality of life and presence of depression/anxiety were determined using validated questionnaires. Disease activity and functional disability in RA patients was assessed using the Disease Activity Score 28-3 and Health Assessment Questionnaire, respectively. Results. FM was present in 15% of patients with RA compared to 2.5% of controls in the North Indian population. RA patients with FM did not differ from those without FM in terms of age, gender, current disease-modifying agents, or steroid use. RA patients with FM had higher disease activity and worse functional disability. The number of tender and swollen joints was higher in patients with FM, but correlated poorly with each other. RA patients with FM had higher pain and fatigue scores but were not different in the quality of life or mood. Conclusion. FM is more common in North Indian patients with RA compared to controls. It adversely affects the pain and fatigue felt by RA patients. Disease activity and FM influence each other.

Long-term outcome of lupus nephritis in Asian Indians.

There are sparse data on outcome of lupus nephritis from developing countries. This study looks at outcome in Asian Indians.

Leprosy revealed in a rheumatology clinic: A case series

Leprosy classically presents with cutaneous and neural involvement. Rheumatological manifestations are frequent, although often under‐recognized. At times, these may present to a rheumatology clinic prior to the diagnosis of leprosy. Herein, we present our experience with patients referred with various rheumatological disorders who were subsequently diagnosed as having leprosy.

Juvenile dermatomyositis at a tertiary care hospital: is there any change in the last decade?

Juvenile dermatomyositis (JDM) is a rare multisystem disorder of childhood primarily involving the skeletal muscles and skin.

Massive ascites as a presenting feature of lupus

Dear Editor, Inflammation of the serous membranes (pericardium, pleural and peritoneum) is referred to as serositis. It may lead to pain, fluid accumulation, adhesion and even fibrosis. The prevalence of serositis in lupus varies depending on the definition used for the diagnosis of serositis, patient selection or whether it has been specifically looked for. Pleural and pericardial involvement is common. The exact prevalence of systemic lupus erythematosus (SLE)-related peritonitis is not known. Peritonitis in lupus may be diseaserelated or due to mesenteric vasculitis, malabsorption, intestinal pseudo-obstruction, cholecystitis, intestinal perforation, appendicitis or pancreatitis. Besides abdominal causes, ascites may also be caused by hypoalbuminemia, right heart failure, Budd-Chiari syndrome, malignancy and infective pathology. We present a rare case of lupus presenting as serositis with massive ascites. A 26-year-old woman presented with a 2-and-a-half month history of abdominal distension and fever, starting 2 weeks after full-term normal delivery. Abdominal distension was gradually progressive, without pain and needed repeated large-volume paracentesis due to rapid re-accumulation. Fever was low grade, present throughout the day without any focus of infection. She had mild pedal edema and breathlessness on exertion (grades II–III New York Heart Association classification). There was no history of oliguria, facial puffiness or icterus. There was no history of joint pain, skin rash, malar rash, oral ulcers, alopecia, Raynaud’s phenomenon, red eye, chest pain or abdominal pain. She had no history of previous tuberculosis or any other significant medical illnesses. On examination she was afebrile, pulse rate of 100/min, respiratory rate of 20/min and blood pressure of 130/70 mmHg. She had bilateral pitting pedal edema with normal jugular venous pressure. Abdominal examination revealed massive ascites with no distended veins or organomegaly. She also had bilateral pleural effusion. Cardiovascular, nervous system and musculoskeletal system examinations were normal. Investigations revealed: hemoglobin of 12.0 gm/dL, total leukocyte count of 11 000/mm, with 77% polymorphs, 14% lymphocytes and 9% eosinophils, peripheral blood film was normocytic and normochromic, reticulocyte count was 1.0% and platelet count was 311 000/mm. Erytherocyte sedimentation rate was 19 mm and Creactive protein was 8.82 mg/dL. Urine examination revealed trace protein with no cellular casts. Biochemical evaluation revealed serum creatinine of 2.77 mg/ dL, protein of 4.5 gm/dL, albumin of 2.5 gm/dL, alkaline phosphotase of 188 IU/L and bilirubin of 0.29 mg/dL. Creatinine kinase was 54 U/L, aspartate aminotransferase was 16 IU/L, gamma-glutamyl transferase of 21.4 IU/L, potassium 3.5 mEq/L and sodium 130 mEq/L. Chest X-ray revealed bilateral moderate pleural effusion. Ultrasonogram of abdomen showed massive ascites and no organomegaly. Echocardiography revealed normal pericardium, no pericardial effusion and normal ejection fraction. Color Doppler of the arterial and venous systems of the abdomen was normal. Ascitic fluid examination revealed albumin of 1.72 gm% (serum ascites albumin gradient – 1), amylase of 11 IU/L, total count of 220/mm with all cells being lymphocytes. Pleural fluid was similar to ascitic fluid and both were negative for bacteria and acid fast bacilli. Both cultures were sterile and no mycobacterium was grown in the peritoneal fluid after 6 weeks. Further evaluation showed anti-nuclear antibody (ANA) positivity with fine speckled pattern, high titre anti-double-stranded DNA (anti-ds-DNA) antibody of 115.0 IU/mL (normal < 30 IU/mL); extractable nuclear antigen (ENA) screen was negative for antibodies to Ro, La, Sm and nRNP, complement component C3 was 44.9 mg/dL (60–150 mg/dL) and C4 was 6.85 mg/dL (15–25). Twenty-four hour urinary protein excretion was 1.05 gm. Thus a diagnosis of SLE was made as the patient had polyserositis, ANA positivity, renal involvement, high anti-dsDNA antibody titers and low complements. Renal biopsy was not performed due to presence of massive ascites. The patient was managed with prednisolone 1 mg/kg, hydroxychloroquine and supportive management. International Journal of Rheumatic Diseases 2012; 15: e15–e16

Methotrexate-induced pancytopenia: a case series of 46 patients

Methotrexate (MTX) has the potential to cause serious adverse reactions and even mortality. We analyzed the predisposing factors and outcome in patients with MTX‐induced pancytopenia admitted into our unit from 1996 to 2015.

Ipsilateral lung dose volume parameters predict radiation pneumonitis in addition to classical dose volume parameters in locally advanced NSCLC treated with combined modality therapy

Purpose: The purpose was to determine the correlation of clinical factors and lung dose volume parameters with significant radiation pneumonitis (RP) in non-small cell lung cancer patients treated with combined modality therapy. Materials and Methods: Between January 2008 and December 2010, 52 patients of non-small cell lung cancer were treated with combined modality therapy with radical intent. Radiation pneumonitis was correlated with ipsilateral (V20 ipsi, V5 ipsi and MLD ipsi) and whole lung (V20, V5, and MLD) dose volume parameters. Clinical factors like pulmonary function tests (PFT), site of tumor, planning target volume, and type of treatment were also correlated with incidence of significant pneumonitis. Results: Out of 52 patients, 35.3% developed grade 2 or more pneumonitis. On univariate analysis, factors significantly correlating with radiation pneumonitis were V5 (P = 0.002), V5 ipsi (P = 0.000), V20 (P = 0.019), V20 ipsi (P = 0.004), MLD (P = 0.008) and MLD ipsi (P = 0.008). On multivariate analysis, V5 ipsi was retained as the most significant factor. Concurrent chemoradiation caused significantly more RP than neoadjuvant chemoradiation (P = 0.004). A cutoff of 65% for V5 ipsi had a sensitivity of 65% and a specificity of 91%. Conclusion: The correlation between pneumonitis and dosimetric constraints has been validated. Adding ipsilateral V20, V5, and MLD to the classical total lung constraints identifies patients likely to develop pulmonary toxicity in patients undergoing chemoradiation.

Adult onset Still's disease: experience from a tertiary care rheumatology unit.

Adult‐onset Stills disease (AOSD) is a rare chronic inflammatory disorder presenting with prolonged fever and polyarthritis.

SLE presenting as migratory arthritis, chorea and nephritis

We present a case of a 12-year-old girl who presented with migratory arthritis, chorea and ascites. She was diagnosed to have systemic lupus erythematosus (SLE) and subsequently responded to immunosuppressive therapy. She had been misdiagnosed earlier as having rheumatic fever. Our case highlights the fact that SLE should be considered in the differential diagnosis of a patient with migratory arthritis & chorea. Generally, chorea in SLE is immune-mediated rather than due to ischemia and has good prognosis.