Pachydermodactyly, mimicker of rheumatoid hands, presents in a patient with Tuberous Sclerosis.

Pachydermodactyly (PDD) is a rare benign condition characterized by painless soft tissue swelling of small joints of hands. The most common presentation is bilateral Symmetrical swelling of proximal interphalangeal and metacarpophalangeal joint similar to Rheumatoid arthritis. The etiology of this disease is unknown, and it sometimes can coexist with other diseases. We present here a case of PDD coexisting with Tuberous Sclerosis, an autosomal dominant genetic disorder characterized by of formation of multiple benign multisystem tumors.

Takayasu Arteritis: Pattern Of Clinical And Radiological Features, Experience From Pakistan.

BACKGROUND: Takayasu arteritis (TKA) is a rare large vessel vasculitis occurring in young adults of less than 50 year of age. We analyse the clinical, radiological features, and treatment regimens in Pakistani patients presenting to a tertiary care center. METHODS: A retrospective cross-sectional analysis of TKA patients done at the Rheumatology department of Fatima Memorial Hospital. A comprehensive evaluation of clinical, laboratory, radiographic features and treatment regimens was carried out. RESULTS: A consecutive cohort of 18 patients, with 13 patients (72%) of female gender was studied. Mean age of the cohort was 35.94±2.7 years. A mean delay of 2.32±0.43 years between symptoms and final diagnosis was reported, attributed to alternate diagnosis in 57.1% and late presentation in 42.8% cases. Limb claudication (44.4%), absent pulses (38.9%), were the common initial manifestation. Hypertension (61.5%), blood pressure discrepancy between arms (88.9%) and bruit (72.2%) over major vessels were common systemic features. As per angiographic classification, Type V (44.4%), and Type I (33.3%), were most common pattern of disease in the cohort. Subclavian artery (72.2%), renal artery (33.3%), iliofemoral arteries (27.8%), and coronary artery involvement (16.7%) were the common lesions. Coronary artery lesion was higher in females (p=0.52) while renal artery lesion in males (p=0.27). There was no statistically significant difference in involvement of vessels according to gender (p >0.05). CONCLUSIONS: Type V and Type I are the common pattern of TKA. Limb claudication was the most common initial manifestation. Renal artery involvement was seen more commonly in males while coronary artery involvement more commonly in females.

Association of systemic lupus erythematosus disease activity index score with clinical and laboratory parameters in pediatric onset systemic lupus erythematosus.

OBJECTIVE: To determine the association of systemic lupus erythematosus disease activity index (SLEDAI) score in pediatric onset SLE (p-SLE) with clinical and laboratory parameters. METHODS: This cross sectional observational study was conducted at Division of Rheumatology, Fatima Memorial Hospital, Lahore from November 2018 to January 2019. Total 23 patients diagnosed with p-SLE having onset of symptoms at ≤ 18 years of age, irrespective of their current age at presentation, of either gender, fulfilling criteria of 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria were enrolled. Patients' clinical symptoms and laboratory parameters were reviewed, SLEDAI scores were calculated. Collected Data were entered in proforma and analyzed on SPSS version 23. RESULTS: There were 91.3% females. Mean age at diagnosis was 11years ± 4years. At presentation patients had hematological involvement 69.6% followed by mucocutaneous symptoms 65.2% and renal involvement 21.6%. ANA by IFA was positive in all, while anti-ds-DNA was positive in 78.3% patients. SLEDAI score was ≥6 in 87% patients, average SLEDAI score was higher in patients with renal involvement (p=0.06). Elevated ESR (r=0.48, p=0.02), Anti-dsDNA (r=0.44, p=0.05) and low complement levels (p=0.03) were significantly positively correlated, while hemoglobin (r= -0.43, p=0.04) was negatively correlated with the SLEDAI score. CONCLUSION: In this study, patients with Lupus Nephritis had high SLEDAI scores. Elevated Anti-dsDNA titer, ESR, low complement levels and hemoglobin were significantly associated with high SLEDAI scores. We recommend that SLEDAI score should be calculated in p-SLE patients for stringent disease monitoring and treatment.