Relapsing polychondritis: clinical presentations, disease activity and outcomes.

BACKGROUND: Relapsing polychondritis is a rare disease characterised by inflammation of cartilaginous and proteoglycan rich structures. As there are only a few published single centre case series from all across the world, we describe our experience with 26 patients at a tertiary centre in north India. METHODS: A retrospective study with all patients meeting Damiani and Levine's modification of McAdam's diagnostic criteria. Clinical details, investigations, disease activity assessment [(Relapsing Polychondritis Disease Activity Index (RPDAI)], treatment and outcomes were recorded. RESULTS: Ten men and sixteen women (median age 45 years) met the diagnostic criteria. Auricular chondritis (96%), arthritis (54%), hearing impairment (42%), ocular (42%), dermal (26%), cardiovascular (11%) and laryngotracheal involvement (11%) characterized the clinical presentations. The median RPDAI was 31 (range 9-66). Two patients died during observation. Overall survival was 92.3% (median survival 13.5 years). CONCLUSIONS: Apart from reduced laryngotracheal involvement, RP in India was clinically similar to recorded patterns elsewhere.

Successful treatment of aortic root dilatation in a patient with relapsing polychondritis.

Cardiovascular involvement occurring in 20-30% patients is the second most common cause of mortality in patients with relapsing polychondritis. Aortic insufficiency occurs as a result of aortic root dilatation rather than primary valvular involvement. We are reporting a patient of relapsing polychondritis with aortic root dilatation, in whom institution of early and aggressive therapy successfully prevented the progression of aortic insufficiency.

Tubercular serpiginous-like choroiditis presenting as multifocal serpiginoid choroiditis.

PURPOSE: To describe the clinical features, course, and outcome in tubercular serpiginous-like choroiditis. DESIGN: Retrospective cohort study. PARTICIPANTS: A total of 105 patients (141 eyes) between May 2002 and July 2010. METHODS: Patients had the following inclusion criteria: (1) complete clinical records and digital fundus images at baseline and follow-up visits, (2) positive tuberculin skin test or QuantiFERON-TB Gold (Cellestis International Pty Ltd. Chadstone, Victoria, Australia) test result, (3) active serpiginous-like choroiditis in at least 1 eye, (4) all known causes of infectious (except tuberculosis) and noninfectious uveitis ruled out, and (5) a minimum of 9 months of follow-up from initiation of treatment that included antitubercular therapy (ATT) with oral corticosteroids (93 patients) or corticosteroids alone (12 patients). MAIN OUTCOME MEASURES: Clinical characteristics and evolution of choroiditis lesions from the acute to healed stage, recurrence, visual outcome, and complications. RESULTS: Mean age was 33 ± 9.3 years (range, 12-54 years; 75 male and 30 female patients). Serpiginous-like choroiditis was bilateral (at least 1 eye active) in 66 patients (62.9%). Of 171 affected eyes, 141 (82.45%) had active lesions at presentation. Of 141 eyes, 115 (81.56%) showed vitreous inflammation. Lesions were multifocal in 133 eyes (94.3%), were noncontiguous to optic disc in 122 eyes (86.52%), and involved the macula in 125 eyes (88.65%). Of patients receiving ATT, all showed resolution of lesions and 9 (9.7%) developed recurrences (median follow-up, 21 months). In addition, 12 patients (12.9%) showed continued progression over a median 3.5 weeks after initiation of therapy. Of 12 patients treated with corticosteroids alone, none showed progression but 9 (75%) developed recurrence (median, 26.5 months). Final visual acuity of ≥ 6/12 was achieved in 108 eyes (76.60%) versus 72 eyes (51.06%) before treatment. Fovea was spared in 95 of 125 eyes (76%) with macular involvement. Five eyes (3.5%) developed choroidal neovascular membrane. CONCLUSIONS: Tubercular serpiginous-like choroiditis presented as multifocal serpiginoid choroiditis affecting predominantly young to middle-aged men. It was frequently bilateral with vitreous inflammation and characterized by multifocal lesions that were noncontiguous to the optic disc and showed serpiginoid spread. Antitubercular therapy significantly reduced recurrences. Lesions responded to combined antitubercular and steroid therapy, usually spared fovea, and had a good final visual acuity.

Causes of DMARD withdrawal following ADR within 6 months of initiation among Indian rheumatoid arthritis patients.

The present study was conducted in Indian rheumatoid arthritis (RA) patients prescribed disease-modifying anti-rheumatic drugs (DMARDs) to determine the incidence and type of adverse drug reactions (ADRs) leading to their withdrawal in the initial 6 months of therapy. This was considered important as pharmacogenetic variations in the pattern of RA in different populations and genetic differences in efficacy and safety to drugs demand separate studies to be conducted in different populations. Hospital records were used to identify 1,000 consecutive patients with RA fulfilling the American College of Rheumatology criteria and having at least 6-month follow-up. Age, gender, duration of arthritis, drug usage and ADR-related drug withdrawal were recorded from the charts. Most of the patients were put on single DMARD. Combined use of DMARD was less frequent and non-use of DMARD was common; however, disease control was good. The commonest DMARD used in our hospital was hydroxychloroquine 444 (44%) and the commonest combination used was methotrexate with hydroxychloroquine by 55 (6%). Sulphasalazine use showed preference to young and males. Supportive drugs used were NSAIDs by 883 (88%), corticosteroids by 646 (65%), paracetamol by 594 (59%) and amitriptyline by 88 (9%). Incidence of ADR-related DMARD withdrawal was maximum with leflunomide 2/15 (13.33%) followed by methotrexate 9/116 (7.76%), sulphasalazine 6/185 (3.24%), chloroquine 3/131 (2.29%) and hydroxychloroquine 8/444 (1.8%). Severity and symptomatology of disease, genetic pattern of patients, financial status, previous experience of the clinicians and patients, availability of drugs, patient expectations and compliance were the main factors that lead to a difference in pattern of therapy in our patients compared to other population.

Ocular signs predictive of tubercular uveitis.

PURPOSE: To determine ocular signs predictive of tubercular uveitis. DESIGN: Retrospective, nonrandomized, comparative interventional case study. METHODS: Three hundred eighty-six patients with active uveitis were treated at a tertiary care single-center uveitis practice. Uveitis was presumed to be tubercular in patients who showed evidence of latent or manifest tuberculosis without any other known cause and who did not show recurrence of uveitis after 12 months of antitubercular therapy. One hundred eighty-two patients who thus obtained clinical diagnoses of presumed tubercular uveitis were enrolled in group A. Two hundred four patients with uveitis resulting from a nontubercular cause were enrolled in group B. Patients were monitored for the presence of types of keratic precipitates (mutton fat or fine), posterior synechiae (broad based or filiform), iris nodules, snowballs, snow banking, vasculitis (with or without choroiditis), serpiginous-like choroiditis, and other types of posterior uveitis (choroidal abscess, retinochoroiditis, or exudative retinal detachment) which were compared between the 2 groups. Statistical analysis was carried out at a 5% level of significance. The main outcome measures were clinical signs significantly associated with tubercular uveitis. RESULTS: Broad-based posterior synechiae, retinal vasculitis with or without choroiditis, and serpiginous-like choroiditis were seen significantly more commonly in patients with tubercular uveitis. Filiform posterior synechiae were more frequent in eyes with nontubercular uveitis. CONCLUSIONS: Broad-based posterior synechiae, retinal vasculitis with or without choroiditis, and serpiginous-like choroiditis in patients with latent or manifest tuberculosis in tuberculosis-endemic areas are suggestive of a tubercular cause of uveitis and merit specific treatment.

Clinical characteristics and predictors of mortality in 67 patients with primary pyomyositis: a study from North India.

Primary pyomyositis is infection of the skeletal muscles in the absence of adjacent skin, soft tissue, and bone infection. This study was undertaken to look at the spectrum of clinical presentations, therapeutic interventions, and their outcomes and also to evaluate the association of various risk factors with mortality. This was a retrospective study in which the patients admitted with the diagnosis of primary pyomyositis from January 2000 to June 2007 were included. Their demographic details, clinical and laboratory data, Sequential Organ Failure Assessment (SOFA) score at presentation, treatment instituted, complications encountered, and hospital outcome were recorded. Sixty-seven patients (42 males and 25 females) with a diagnosis of primary pyomyositis were included. Median age at the time of presentation was 37 years (interquartile range = 25-50 years). Common presenting symptoms were myalgias [50 (74.62%)] and fever [49 (73.13%)]. Twenty-six patients had underlying predisposing medical conditions. The commonest muscle group involved was iliopsoas muscles in 31 (46.26%) patients. Methicillin-sensitive Staphylococcus aureus was the commonest organism isolated from the pus. Twenty-eight patients developed sepsis and seven died. On univariate analysis, there was a statistically significant association between higher SOFA score, lower Glasgow coma scale, higher pulse rate, lower blood pressure, raised blood urea, raised serum creatinine, higher serum glutamic pyruvate transaminase, raised total bilirubin at presentation, and development of sepsis during hospital stay with mortality. In our study, the patients were seen almost a decade later than those seen in other studies from the region. Evidence of organ dysfunction at presentation and sepsis was associated with increased mortality.

High-dose prednisolone and bolus cyclophosphamide in interstitial lung disease associated with systemic sclerosis: a prospective open study.

AIM: Currently, therapy for interstitial lung disease in patients with systemic sclerosis is unsatisfactory. A prospective open label study was conducted in a North Indian tertiary Institute to assess the efficacy of intermittent pulse cyclophosphamide (CYC) and high-dose prednisolone in systemic sclerosis (SSc)-related interstitial lung disease (ILD). METHODS: Consecutive patients with SSc and ILD, diagnosed on spirometry, carbon monoxide diffusing capacity (DLCO) and high-resolution computed tomography (HRCT) scan were treated. Pulmonary function tests were carried out at baseline and after 6 months. Patients received oral prednisolone 1 mg/kg body weight initially, with tapering to a dose of 7.5 mg/day was reached. Monthly CYC pulses were given for 6 months followed by 3-monthly maintenance pulses. CYC was discontinued in patients with declining pulmonary function, adverse effects or static disease after 6 months. RESULTS: Average disease duration of 36 patients was 59.78 +/- 63.22 months. Seven patients improved (forced vital capacity [FVC] increase 10% or DLCO increase 15%), five deteriorated (FVC decline 10% or DLCO decline 15%) and 24 had stable disease. Thus, 31 out of 36 patients either improved or had static lung disease. Mean FVC (% of predicted) improved by 4.16% over 6 months (P = 0.069). Mean DLCO (% of predicted) improved by 5.66% (P = 0.27). Average % of predicted DLCO at baseline was 39%. CONCLUSION: High-dose prednisolone with pulse CYC can either improve or stabilize lung functions in patients with severe systemic sclerosis lung disease irrespective of presence of ground glass appearance on HRCT.

Role of anti-tubercular therapy in uveitis with latent/manifest tuberculosis.

PURPOSE: To assess the role of anti-tubercular therapy in uveitis with latent/manifest tuberculosis (TB). DESIGN: Retrospective, interventional case series. METHODS: A total of 360 patients from uveitis clinic with following inclusion criteria were studied: 1) complete clinical records of visual acuity, slit-lamp biomicroscopic examination, intraocular pressure, complications if any, and treatment records at the baseline and at all follow-up visits; 2) a documented positive tuberculin skin test (10 mm of induration or more) at 48 to 72 hours; 3) evidence of active uveitis, i.e., cellular reaction in the anterior chamber with or without keratic precipitates, and/or active vitreous inflammation, retinal vasculitis, choroiditis, or neuroretinitis; 4) all known causes of infectious uveitis except TB and known noninfectious uveitic syndromes ruled out; and 5) a minimum one year of follow-up from the initiation of treatment. Of these, 216 patients (Group A) received four-drug anti-tubercular therapy and corticosteroids, and 144 patients (Group B) received corticosteroids alone. The main outcome measure was recurrence of inflammation after minimum six months of initiating treatment in each group. RESULTS: Recurrences reduced significantly (P < .001) in Group A (15.74%) as compared to Group B (46.53%) over a median follow-up of 24 and 31 months, respectively. The patients treated with anti-tubercular therapy with corticosteroids had decreased risk of developing recurrence of uveitis by approximately two-thirds as compared to those treated with corticosteroids alone. CONCLUSION: Addition of anti-tubercular therapy to corticosteroids in uveitis patients with latent/manifest TB led to significant reduction in recurrences of uveitis.

Serum transferrin receptor-ferritin index shows concomitant iron deficiency anemia and anemia of chronic disease is common in patients with rheumatoid arthritis in north India.

Anemia is a frequent cause of morbidity in patients with rheumatoid arthritis (RA). We studied the prevalence of anemia of chronic disorders (ACD) and ACD with coexistent iron deficiency anemia (IDA) in patients with RA using sTfR/log ferritin ratio (sTfR - F index). Complete blood counts, percent transferrin saturation, serum ferritin, sTfR, sTfR-F index measurements were carried out in 100 anemic RA patients. Twenty-five IDA subjects without any other illness and 25 age- and sex-matched normal controls were studied. Prevalence of anemia in RA patients was 50.5%. Patients with sTfR-F index value < 1.5 were classified as pure ACD and patients with sTfR-F index value> 1.5 were classified as ACD with coexistent IDA. Using these criteria, 20% patients were found to have pure ACD and 80% patients had coexistent ACD and IDA. In the normal control group, sTfR-F index was found to be 0.16-1.8. We found that sTfR-F index can clearly distinguish IDA control cases and normal subjects with no overlap in the range of sTfR-F index.

Cryptococcal meningitis in HIV infected: experience from a North Indian tertiary center.

BACKGROUND: Cryptococcal meningitis is a common opportunistic infection in Human Immunodeficiency Virus (HIV)-infected individuals. There is little information specifically addressing cryptococcal meningitis in HIV-infected patients from North India. AIMS: To determine clinical presentation, hospital course, response to treatment, complications developed, in-hospital mortality, any recurrence of cryptococcal meningitis and reasons of recurrence during follow-up. SETTINGS AND DESIGN: A retrospective observational study undertaken in a large tertiary care center. PATIENTS AND METHODS: Patient's demographic data, presenting clinical symptomatology, physical findings, laboratory parameters, cerebrospinal fluid (CSF) examination findings, side-effects of treatment, development of any complications and hospital outcome were analyzed. During follow-up any recurrence of cryptococcal meningitis, possible reasons of recurrence, type of treatment received, complications developed and outcome was recorded as well. RESULTS: Forty patients diagnosed to have cryptococcal meningitis were analyzed. Twenty-two (55%) patients had acute/ subacute presentation. Thirty-six (90%) patients presented with headache and 18 (45%) had altered sensorium. Twenty (50%) patients had no cells in the CSF. Hypoglycorrhchia was seen in 30 (75%) patients. Cryptococcal meningitis was the first acquired immune deficiency syndrome (AIDS)-defining illness in 30 (75%) patients. Thirty-five patients developed some adverse effects to amphotericin-B. Thirty-three patients improved with treatment while three patients died. Four patients had recurrence of cryptococcal meningitis within six months of first episode. Non-compliance of fluconazole therapy was the reason for recurrence in all of these patients. CONCLUSIONS: Cryptococcal meningitis is a common initial AIDS-defining illness. Acute and/or subacute presentation of cryptococcal meningitis is not uncommon in HIV-infected individuals. An early diagnosis of HIV infection might reduce the incidence of this infection.

Phrenic nerve palsy in a patient of Churg Strauss syndrome and mononeuritis multiplex.

Neurological manifestations like mononeuritis multiplex are seen commonly in patients with Churg Strauss syndrome. Cranial nerve involvement and central nervous system involvement are also reported, although infrequently. Phrenic nerve involvement has not been reported so far. We report a patient with Churg Strauss syndrome who presented with mononeuritis multiplex and developed left-sided phrenic nerve palsy subsequently.

HLA typing in Vogt-Koyanagi-Harada syndrome in North Indian patients.

PURPOSE: To report the HLA profile of VKH patients from India. METHOD: Forty-one patients and 50 controls were studied. Phenotyping using a lymphocytotoxicity assay was done for HLA-A and -B. DNA-based sequence-specific low resolution typing was done for HLA-DR and -DQ loci. RESULTS: HLA-A9 was over-represented in the patient population (p = 0.01), whereas HLA-A11 (p = 0.03) and HLA-DRB1*13 (p = 0.007) were found to be underrepresented. The frequency of HLA-DRB1*04 was 14.6% and 10% in the patient population and controls, respectively. The HLA-DQ frequencies did not differ significantly between patients and controls. CONCLUSION: Unlike that reported in most populations, we did not find a significant association between HLA-DRB1*04 and our patient population.

Acute tubulo-interstitial nephritis leading to acute renal failure following multiple hornet stings.

BACKGROUND: Hornet stings are generally associated with local and occasionally anaphylactic reactions. Rarely systemic complications like acute renal failure can occur following multiple stings. Renal failure is usually due to development of acute tubular necrosis as a result of intravascular haemolysis, rhabdomyolysis or shock. Rarely it can be following development of acute tubulo-interstitial nephritis. CASE PRESENTATION: We describe a young male, who was stung on face, head, shoulders and upper limbs by multiple hornets (Vespa orientalis). He developed acute renal failure as a result of acute tubulo-interstitial nephritis and responded to steroids. CONCLUSION: Rare causes of acute renal failure like tubulo-interstitial nephritis should be considered in a patient with persistent oliguria and azotemia following multiple hornet stings. Renal biopsy should be undertaken early, as institution of steroid therapy may help in recovery of renal function.

Idiopathic endogenous lipoid pneumonia.

Lipoid pneumonia is a rare pulmonary disorder having no classical radiological appearance. We report a 33-year-old male, ex-smoker who was referred to us with history of cough, mild mucoid expectoration and progressively increasing dyspnoea since one year. He was investigated at local hospital and was treated with 30 mg prednisolone per day for 6 months for sarcoidosis without any response. On examination, he was normal except for fine basal crepitations in chest. Pulmonary function test (PFT) revealed mild airway obstruction. High resolution computerised tomographic scan (HRCT scan) revealed bilateral reticulonodular shadows and bronchiectasis in lower zones. Open lung biopsy revealed lipoid pneumonia. As there was no history of nasal distillation of oils, it was diagnosed to be idiopathic. The relevant literature is reviewed.

Leflunomide reduces nitric oxide production in patients with active rheumatoid arthritis.

OBJECTIVES: Leflunomide is an immunomodulatory agent that was recently approved for the treatment of rheumatoid arthritis (RA). The mechanism of action is not fully understood. Nitric oxide (NO) plays an important role in the pathogenesis of RA. Leflunomide has been shown to cause cell specific inhibition of inducible nitric oxide synthase (iNOS) activation in animal models. We carried out this study to determine if there was alteration in NO production in patients with RA. METHODS: An 8-week open label study was carried out on patients with adult onset active RA. We measured levels of nitrite and citrulline spectrophotometrically as surrogate markers of NO production. Within-patient serum levels of nitrite and citrulline were compared with leflunomide therapy at three points of time (at 0, 4 and 8 weeks of therapy). RESULTS: Thirty-three patients with active RA were enrolled for this study. These patients were a subset of 63 individuals who are studied for clinical efficacy of leflunomide. Three patients were lost to follow-up. Median nitrite levels were 817.2 (nmol/ml) at the start of therapy and this declined to 440.9 nmol/ml and 301.1 nmol/ml at 4 and 8 weeks of therapy. Median citrulline levels were 649.3 nmol/ml at the start of the study, which declined to 549.2 nmol/ml and 485.4 nmol/ml at 4 and 8 weeks, respectively. Statistically significant decrease in median values for serum nitrite and citrulline levels was documented after 4 weeks of leflunomide therapy (p<0.01), which was sustained at 8 weeks (p<0.01), although there was no further fall between 4 and 8 weeks (p>0.1). CONCLUSIONS: Leflunomide inhibits nitric oxide production in patients with active RA. Inhibition of NO synthesis may be one of the mechanisms responsible for the immunomodulatory activity of leflunomide.