Sumantro Mondal

Development of a colour Doppler ultrasound scoring system in patients of Takayasu’s arteritis and its correlation with clinical activity score (ITAS 2010)

OBJECTIVES The objectives of this study were to develop a scoring system with colour Doppler ultrasound (CDUS) in patients with Takayasus arteritis, to correlate the CDUS score with the Indian Takayasus Activity Score (ITAS) and to assess the degree of agreement between CDUS and angiogram in the diagnosis of Takayasus arteritis. METHODS Nineteen angiographically confirmed Takayasus arteritis patients fulfilling three or more of the 1990 ACR criteria were evaluated. Their ITASs were recorded. A CDUS scoring system, CDUS-Kolkata (CDUS-K), was devised based on the presence of stenosis and altered flow patterns. It was then correlated with the ITAS. An inter-rater agreement analysis was done between the CDUS-K scores and angiographic scores in selected arterial sites. RESULTS We found a significant degree of correlation between the ITAS and the CDUS-K score (r = 0.7144, 95% CI 0.3852, 0.8823, P = 0.0006). A high degree of correlation was found between the CDUS-K and angiographic scores in the selected arterial sites (κ-value = 0.725 on inter-rater agreement analysis). CONCLUSION CDUS imaging may be used as an objective tool for assessing disease severity in Takayasus arteritis. Development of a CDUS-K scoring system would be a supplementary tool to clinical scoring and the ITAS. CDUS imaging could be a cost-effective, non-invasive and reliable substitute for angiogram, especially for follow-up in Takayasus arteritis patients.

Ultrasound is a useful adjunct in diagnosis of eosinophilic fasciitis

This 28-year-old male presented with progressive skin tightening and limited joint mobility for 11 months, involving legs up to mid-shin and forearms up to elbows, preceded by painless swelling involving affected regions, sparing fingers and toes. Systemic features were absent. There was peripheral eosinophilia (leucocytes: 11 600/ml, eosinophils 52%, absolute eosinophil count: 6032/ml), raised acute-phase reactants and hypergammaglobulinaemia. Nail-fold capillaroscopy was noncontributory. RP, digital ulcers, dysphagia, reflux and pulmonary arterial hypertension were absent. ANA was positive, and Scl70 and anticentromere antibodies were negative. High-resolution US of legs (Fig. 1A and C) using an 18-MHz linear array transducer showed thickening and altered echointensities afflicting the fascia, alongside deranged echotexture of subcutaneous fat (Fig. 1B is from a healthy person for comparison). MRI of legs (Fig. 1D) showed thickening of fascia, along with increased contrast enhancement of the myofascial plane, corroborating US findings. He was diagnosed as having eosinophilic fasciitis (EF) and put on prednisolone. Apart from histology, MRI is also useful for the diagnosis of EF [1]. The role of US elastography has been studied in scleroderma, and has demonstrated reduction of strain in the dermis of the forearm due to loss of elasticity [2]. Here we present a case of EF characterized ultrasonographically with MRI corroboration.

Ultrasonography as a useful modality for documenting sacroiliitis in radiographically negative inflammatory back pain: a comparative evaluation with MRI

OBJECTIVES The aims of this study were to identify and characterize features of sacroiliitis in patients with non-radiographic inflammatory low back pain by ultrasonography (USG) and to correlate the findings with that of MRI. METHODS MRI and USG of SI joints were performed on 29 patients who fulfilled the definition of inflammatory low back pain according to the Assessment of SpondyloArthritis International Society 2009 criteria for axial SpA but were X-ray negative for sacroiliitis. Increased vascularity, low resistive index (RI) and hyperechogenicity of the joint space were considered USG features of sacroiliitis. The findings were compared with those of 32 controls. USG features of sacroiliitis were compared with MRI by κ statistics. RESULTS Receiver operating characteristic analysis revealed cut-off values for flow signals and RI of 3 and 0.605, respectively. There was a significant difference in the number of flow signals, RI and echogenicity of the SI joint between MRI-proven cases and controls. The Cohens κ for flow signals, RI and hyperechogenicity when compared with MRI were 0.816 (95% CI 0.676, 0.937) and 0.821 (95% CI 0.662, 0.965) and 0.403 (95% CI 0.108, 0.695). Taking both flow signals and RI parameters as criteria for determining sacroiliitis, comparison with MRI returned a κ of 0.816 (95% CI 0.601, 0.963). CONCLUSION Three or more flow signals and a RI ≤0.605 can be applied as USG criteria for sacroiliitis. USG can be a cost-effective and non-inferior modality compared with MRI in documenting sacroiliitis in early SpA.

Shrinking lung syndrome in systemic lupus erythematosus successfully treated with rituximab

Learning point for clinicians Shrinking lung syndrome (SLS) is a systemic lupus erythematosus (SLE) specific complication. Restrictive respiratory disease, normal lung parenchyma and elevated hemidiaphragms on chest X-ray are characteristic. Diaphragmatic dysfunction, pleuritis and anti-Ro antibody are known associations.1–3 Prognosis is generally favourable on corticosteroids, but exceptions do occur. We report a patient with SLS, presenting with acute onset severe respiratory embarrassment, who was difficult to treat with corticosteroids and cyclophosphamide. A 38-year-old female presented with proximal muscle weakness, fever, arthritis and photosensitive skin rashes for 3 weeks and exertional shortness of …

Unilateral sternocostoclavicular hyperostosis in a patient with ankylosing spondylitis: A case report with color Doppler ultrasonogram findings

Sternocostoclavicular hyperostosis is a chronic inflammatory disorder affecting the sternoclavicular joint and upper ribs. There is a strong association with seronegative spondyloarthropathy in which bilateral involvement is common. Ultrasonography and Color Doppler findings of this entity have not been described previously, to the best of our knowledge. We describe the findings in a patient of ankylosing spondylitis who was referred for unilateral sternoclavicular joint swelling.

Posterior reversible encephalopathy syndrome in a patient with lupus nephritis on rituximab therapy: a challenge to find the offender

Sir, Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological entity characterized by seizure, altered sensorium, visual disturbance and nausea, and vomiting, along with characteristic neuroimaging findings. Magnetic resonance imaging (MRI) often shows bilateral, symmetric areas of white matter edema predominantly in occipital and posterior parietal lobes. Autoimmune diseases are associated with 8–10% of PRES cases. PRES has been reported in patients with systemic lupus erythematosus (SLE). On the other hand, drugs, chiefly chemotherapeutic agents, immunosuppressive agent, and monoclonal antibodies including rituximab, are also considered as possible etiological agents. We report a patient of lupus nephritis who developed PRES following third dose of rituximab infusion. A 17-year-old female was admitted with a flare of lupus nephritis. She was diagnosed as SLE with nephritis (class IV-A) about 21⁄2 years previously and received cyclophosphamide according to NIH protocol followed by maintenance therapy with mycophenolate mofetil along with corticosteroid. She responded well but developed the current episode of nephritis flare while on regular maintenance therapy. Urine analysis showed active sediments with nephrotic range proteinuria (9.2 g/24 hours). She was started with rituximab (375mg/m of body surface area/week). Twelve hours after the third dose of rituximab she developed repeated attacks of convulsions, visual disturbances in the form of blurring of vision, and she was stuporous (Grady coma scale II and Glasgow coma scale 9). Blood pressure was 130/98mmHg. Plantar reflexes were extensor bilaterally. No focal neurological signs were present. Fundoscopy and cerebrospinal fluid studies were normal. MRI of the brain was consistent with a diagnosis of PRES (Figure 1). She responded to conservative management and follow-up MRI after four weeks was normal. She was not given the fourth dose of rituximab. Approximate prevalence of PRES among SLE patients is 0.69%. PRES is found more commonly among Asian patients. Most of the patients are female with highly active disease and renal involvement. Most of them are also hypertensive (91.7%). In an Indian cohort of 13 SLE patients, six had PRES as a part of their initial presentation of lupus. PRES is more common among young lupus patients in the early part of the disease. Almost 90% patients show full recovery. Our patient had most of these clinical parameters implicated in the development of PRES except for significant hypertension. PRES following rituximab therapy has also been documented in the literature. Mavragani et al. reported PRES in a lupus nephritis patient following a third dose of rituximab infusion. The exact pathogenesis of PRES following rituximab is not definitely known. Hypertensive crisis seen in few patients following rituximab therapy may be an important factor. However, there was no such event in our patient. Endothelial activation by rituximab through CD20 binding may be another possible mechanism. In this patient it was difficult to determine whether PRES was due to disease or drug. However, absence of significant hypertension, normal fundoscopic finding, and a clear temporal association between symptom development and rituximab infusion prompted us to withhold rituximab. We report this case as an example of a

Sterno Clavicular Joint Osteoarthritis in a Patient With Rheumatoid Arthritis: A Case Report With Classical Sonographic Features

Osteoarthritis of the sternoclavicular joint (SCJ) is a common entity. Plain radiographs and computed tomographic scans are the modalities commonly used for diagnosis of SCJ osteoarthritis. Classical imaging findings are joint space narrowing, marginal sclerosis, and osteophyte formation. Widespread application of ultrasonography (US) in the evaluation of multiple musculoskeletal pathologies has been made recently. However, the sonographic features of SCJ osteoarthritis have not been reported. We report a case of unilateral SCJ osteoarthritis in a patient with rheumatoid arthritis (RA) and describe its typical sonographic features.

Interstitial Lung Disease as Presenting Feature of SLE

Interstitial lung disease (ILD) has been considered as an unusual pulmonary manifestation of systemic lupus erythematosus. Patients of ILD usually present with progressive dry cough and exertional dyspnea. ILD is usually seen in long standing SLE patients and follows a chronic course. ILD as the initial manifestation of SLE is very rare. Our middle aged female patient presented with pulmonary symptoms, renal impairment and arthralgia. She was finally diagnosed as having SLE by relevant autoantibody profile and renal biopsy along with HRCT proven ILD.

Middle Cerebral Artery Dissection Presenting with Intracerebral and Subarachnoid Hemorrhage

Dissecting aneurysm of the intracranial arteries is uncommon and isolated dissection of middle cerebral artery (MCA) is much rarer. Cerebral infarction is the usual presentation of MCA dissection in first two decades, hemorrhagic manifestation being much rare. We describe a young male patient who presented with acute onset headache and left sided hemiparesis due to acute intra-parenchymal hemorrhage. Digital subtraction angiography finally revealed dissection involving M2 segment of right MCA as the possible cause of intracerebral hemorrhage in this patient. This type of uncommon presentation of MCA dissection is rarely reported before.

Ultrasonography of Knee Joint in Hemophilia A: What the Eyes Cannot See

Haemophilia is the most common inherited coagulopathy. Approximately 94% patients suffer from joint disability. An imaging modality to detect joint damage can help in monitoring. Ultrasonography (USG) provides a low cost and reliable imaging alternative to magnetic resonance imaging. This study aims at the detection of subclinical knee-joint involvement by USG, in patients with moderate to severe Haemophilia. 27 patients suffering from moderate and severe Haemophilia and 27 age-matched controls were studied. USG of bilateral knee joints was done to analyse cartilage and synovial thickness, synovial vascularity and resistive index of vascular flow along with synovial collection. The relevant clinical parameters (age at diagnosis and study enrolment, severity of haemophilia A, annualized bleeding rates, total number of joint bleeds, spontaneous and provoked bleed, number of episodes treated with factor VIII injection) were noted. The USG findings were correlated to the clinical parameters and subclinical joint bleed detection looked for. 13 patients [18 out of 54 joints (33.33%)] showed increased vascular signals with mean resistive-index (RI) 0.67 (± 0.086; 95% CI: 0.62–0.70). The mean synovial thickness in persons with haemophilia (PwH) was higher than the control subset (p < 0.05 on all counts). The mean cartilage thickness was lower in PwH than in controls. On a subset analysis, there was significant difference between the mean cartilage thickness between moderate PwH and age matched controls (p < 0.0001). 3 patients (11.1%) showed evidence of joint collection (hemarthrosis) despite having no clinical evidence of joint involvement. Through the findings of our study, we do infer that ultrasonography can detect subclinical synovial inflammation and cartilage damage in haemophilia patients that may affect long term articular outcome. It is also a useful modality for detection of sub clinical joint bleed.