Kuntal Bhattacharyya

Adult onset Still's disease with persistent skin lesions complicated by secondary hemophagocytic lymphohistiocytosis

Dear Editor, Here we describe an interesting case of a middle-aged male patient presenting with adult onset Still’s disease (AOSD) complicated by secondary hemophagocytic lymphohistiocytosis (HLH) and generalized dermal and mucosal hyper-pigmentation. AOSD is a rare, inflammatory disease of unknown etiology, affecting primarily young adults, and characterized by high spiking fevers, arthritis and an evanescent, macular, non-pruritic, salmon-colored rash, distributed on the trunk and the extremities. Organomegaly and lymphadenopathy are common associations. Laboratory tests reveal neutrophilic leukocytosis, negative rheumatoid factor (RF) and antinuclear antibodies (ANA), as well as high serum ferritin levels and low serum glycosylated ferritin levels. A small number of AOSD cases complicated by secondary hemophagocytic lymphohistiocytosis (HLH) have been described. Our patient, a 36-year-old man, presented with asymmetric, recurrent, multiple large joint arthritis associated with spikes of high grade fever (> 39°C) for the last 8 months and hyper-pigmentation for 4 months. There was no history of long-term drug intake, photosensitivity, diarrhea or weight loss. Examination revealed pigmented non-pruritic patches and plaques on his chest wall (Fig. 1), dermal and mucosal hyper-pigmentation, anemia, fixed flexion deformity with arthritis in both knees and mild splenomegaly. Examination was notable for absence of lymphadenopathy and hepatomegaly. Initially, investigations included microcytic hypochromic anemia (hemoglobin: 6.9 g/dL), neutrophilic leukocytosis (total leukocyte count: 16 800; neutrophil 78%) and high erythrocyte sedimentation rate (ESR) of 58 mm in the 1st hour, and hypoalbuminemia (2.4 g/dL). He had normal plasma glucose, urea, creatinine, electrolytes and liver enzymes. He had elevated C-reactive protein, and raised serum ferritin (4821 ng/mL; normal: 30–400). Anti nuclear antibody (ANA) and rheumatoid factor (RF) were negative, as was HFE mutation assay. Serum cortisol at 6 a.m. was normal. The patient was not immuno-compromised, and hepatitis B surface antigen and anti-hepatitis C virus were

Serum creatine phosphokinase: a probable marker of severity in organophosphorus poisoning.

Organophosphorus (OP), the commonest agent for poisoning in India due to its easy availability, acts by inhibiting acetylcholinesterase at muscarinic and nicotinic receptors. Erythrocyte cholinesterase (EchE) and plasma cholinesterase (PchE) are reduced in OP poisoning, but their estimation is costly and not regularly performed. There are emerging options for new cheaper biochemical markers in relation to OP poisoning. Serum level of creatine phosphokinase (CPK) is often found to be elevated in OP poisoning. This study was conducted to see if CPK may be used as an alternative of cholinesterase levels in blood to assess the severity of OP poisoning. This was a prospective and observational study. Sixty-three patients of OP poisoning without any prior treatment, presenting within 6 hours, were selected and their clinical severity was categorized according to Peradeniya organophosphorus poisoning (POP) scale. Level of serum CPK, blood EchE and pH were measured following admission, and total dose of atropine (mg) until the final clinical outcome (complete recovery or death) was calculated. Student′s t-test and Pearson′s correlation coefficient was used for the assessment of statistical significance. According to POP scale, clinical severity was mild (score 0-3) in 17 (27%), moderate (score 4-7) in 32 (50.8%) and severe (score 8-11) in 14 (22.2%) patients. Serum CPK, EchE level, blood pH and total atropine dose strongly correlated with clinical severity. Our study recommends serum CPK as an alternative marker.

Incidence and risk factors of immune reconstitution inflammatory syndrome in HIV-TB coinfected patients

UNLABELLED Tuberculosis is one of the leading causes of development of Immune reconstitution inflammatory syndrome (IRIS) in HIV patients receiving antiretroviral therapy (ART). OBJECTIVE To determine the incidence of IRIS in HIV-TB coinfected patients, and to find out the possible risk factors associated with IRIS. MATERIALS AND METHODS Study commenced with 96 patients adhered to standard antitubercular therapy (ATT) and ART without defaulting, and followed up for six months. RESULT The mean (± SD) CD4 count and CD4 percentage at baseline was 59.16 (± 24.63) per mm³ and 4.59% (± 1.73) respectively. Only 18.75% developed IRIS after 57.05 (± 14.12) days of initiation of ART. Extrapulmonary tuberculosis was the most significant factor associated with IRIS (83.33%) than those without IRIS (44.87%) (p = 0.0032). Specifically, tubercular lymphadenitis (38.88%, p = 0.0364) and disseminated tuberculosis (33.33%, p = 0.0217) were significantly associated with IRIS. The other risk factors associated with appearance of IRIS were higher CD4 count (p = 0.0212) at three months after initiation of ART and increment of CD4 count (p = 0.0063) and CD4 percentage (p = 0.0016) during this period. The major manifestations of IRIS were fever (40%), followed by lymphadenitis (38%). The mortality rate in IRIS was not higher than those without IRIS. CONCLUSION Patients with extrapulmonary tuberculosis, especially tubercular lymphadenitis, were more likely to develop IRIS and fever was associated in most of them. Higher increment of CD4 count may indicate development of IRIS in presence of new or worsening tuberculosis lesion.

Musculoskeletal involvement in leprosy

Abstract Introduction Leprosy classically presents with cutaneous and neurological involvement, though musculoskeletal symptoms may be the initial symptom. Methods The study was conducted on 102 patients with leprosy to determine the prevalence and spectrum of rheumatological diseases in this group. Eighty four patients were referred from leprosy clinic and 18 patients presented first in rheumatology clinic with arthritic symptoms and were later diagnosed to have leprosy. Results In the first group, symmetrical polyarthritis involving the large and small joints, similar to rheumatoid arthritis was the most common manifestation. Whereas in the second group, tenosynovitis was the commonest presentation. Rheumatoid factor was positive in 72 (70%) cases. Discussion Variety of musculoskeletal symptoms were found in patients with leprosy and many of them were associated with Lepra reaction.

Study of endothelial dysfunction and its correlation with disease activity in systemic lupus erythematosus patients without conventional cardiovascular risk factors

Abstract Background Endothelial dysfunction is an important cause of cardiovascular morbidity and mortality in patients of systemic lupus erythematosus (SLE), causing atherosclerosis and its related complications, even in the absence of conventional cardiovascular risk factors. Objective The present study was aimed at finding the prevalence of endothelial dysfunction in SLE and its relation with disease activity. Methods Fifty SLE patients without traditional cardiovascular risk factors, and equal number of age and sex matched controls were chosen. Endothelial function was assessed by flow mediated vasodilatation (FMV) on brachial artery using B-mode ultrasonography. Results Mean age of SLE patients was 26.06 ± 6.231 years. Thirty-seven (74%) of the patients were having severe disease activity, defined as systemic lupus erythematosus disease activity index (SLEDAI) >8. We found significantly lower levels of basal brachial artery diameter in SLE patients (0.3499±0.075 cm) compared to controls (0.3826 ± 0.0002cm), as also impaired FMV (2.57 ± 2.32% and 8.7082 ± 1.5776%, respectively). Flow mediated vasodilatation significantly correlated with SLEDAI (r=-0.52) and complement (C3) levels (r=0.33). Conclusion Endothelial dysfunction in SLE correlates with disease activity.

Unravelling the Gordian knot: diagnostic dilemma in an HIV-positive patient with neurological involvement

We report a case of a 40-year-old seropositive-HIV patient with a CD4 count of 120 who presented with fever, severe headache and neck stiffness. Suspecting a case of tubercular meningitis (TBM; as tuberculosis is the commonest opportunistic infection in HIV/AIDS patients in India), a lumbar puncture was performed and a cerebrospinal fluid study revealed TBM. The patient was started on combination antitubercular drug therapy from directly observed treatment, short course (DOTS) (Cat 1 regimen) along with pyridoxine 40 mg/day and adjunctive corticosteroid therapy. However, despite adequate antitubercular therapy for 4 weeks, the patient did not show any improvement in his clinical condition. On the contrary, in the process he developed cytomegalovirus (CMV) retinitis. So we question our learned readers if the coinfection of Mycobacterium tuberculosis and CMV should be implicated for the failure to respond to isolated antitubercular therapy contrary to our expectation.

Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome in a tertiary care hospital: A retrospective analysis

Background Remitting seronegative symmetrical synovitis with pitting edema (RS 3 PE) affects mainly elderly males, characterized by acute onset pitting edema of hands, negative rheumatoid factor and excellent result with short course of low dose corticosteroid therapy. Objective To re-evaluate the clinical, laboratory features, management, and ultimate disease progression of RS 3 PE cases in rheumatology clinic in tertiary care hospital and compare results with previous literature. Methods We selected the patients of RS 3 PE in the Rheumatology clinic, Medical College, Kolkata, between 1 January 2002 and 1 January 2010 and followed them up. Results Among the cases reviewed 23 were female patients and 12 were male patients with mean (SD) age of 54.74 (3.03) and 58.33 (5.33) years, respectively. Oral Hydroxychloroquine was started in all, giving a satisfactory result in 21 patients; rest of the 14 patients needed additional corticosteroid, to alleviate musculoskeletal symptoms. Baseline mean (SD) C-reactive protein and erythrocyte sedimentation rate were 21.63 (2.08) mg/L and 81.34 (9.29) mm/hr, respectively, and reduced to 3.23 (0.95) mg/L and 17.43 (5.55) mm/hr, respectively, following therapy. During follow-up, musculoskeletal symptoms relapsed in 17 patients, seven (20%) of them were found to be associated with malignancy. At the time of last visit during the follow-up period, only four patients developed definite arthropathy (one seronegative rheumatoid arthritis, three undifferentiated spondyloarthropathy), rest of the 31 cases were remitted. Conclusion Regarding RS 3 PE, results of our study showed similarity with previous reports excepting the gender. Hydroxychloroquine has been proved as a good steroid-sparing agent in RS 3 PE. This study supported the association of RS 3 PE with malignancy in the previous literatures.

Assessment of the efficacy of pulse Ibandronate therapy in non-steroidal anti-inflammatory drug refractory ankylosing spondylitis: An open prospective study

Abstract Background Amino-bisphosphonates like Pamidronate, having weak anti-Tumour Necrosis factor (TNF)-alpha property is effective in ankylosing spondylitis (AS). Objective To assess the efficacy of intravenous pulse Ibandronate, another bisphosphonate, in non-steroidal anti-inflammatory drug (NSAID) refractory/intolerant AS, as it is cheaper, requires less frequent dosing and is easily available. Methods Thirty-four patients, diagnosed as AS by Modified New York Criteria, having Bath AS Disease Activity Index (BASDAI) ≥ 4 (i.e. active disease) and NSAID refractory/intolerant, received six doses of injection Ibandronate (3 mg) intravenously, three monthly, for 15 months. Patients with peripheral arthritis were given sulfasalazine along with Ibandronate. Outcome assessed comparing baseline and final BASDAI, Bath AS metrology index (BASMI), Bath AS functional index (BASFI), Bath AS global score (BAS-G), erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Response defined according to assessments in ankylosing spondylitis (ASAS)-20 and BASDAI-50, and, reduction in tender and swollen joint counts in patients with peripheral arthritis. Results Twenty-eight patients received all six doses. Twenty-one (75%) and 23 (82%) patients achieved BASDAI-50 and ASAS-20 responses, respectively. Significant reductions in mean BASDAI (48.36%), BASMI (55.08%), BASFI (47.03%), BASG (47.47%), ESR (49.24%) and CRP (66.38%) were noted after 15 months. Ten patients having peripheral joint involvement had significant decrement in swollen (67.74%) and tender (57.14%) joint counts. Conclusion Intravenous Ibandronate has reasonable efficacy in the treatment of AS.