Klippel-Trenaunay-Weber (KTW) syndrome is a rare syndrome characterized by hemangiomata, varicose veins, and both bony and soft tissue hemihypertrophy. Abdominal viscera affected by ipsilateral hemangiomata include colon, liver, spleen, jejunum, kidney, and liver. We report a case of this syndrome that presented with severe anemia and extensive jeujenal varices without any other significant digestive tract lesions.
Background Systemic lupus erythematosus (SLE) is a rare complex autoimmune disease with a multisystem involvement [1]. Although pulmonary involvement is relatively frequent in adult patients; it has been rarely reported in children with SLE. It may be an initial and/or life-threatening complication [2]. There is a scarcity of published literature on pulmonary function tests (PFTs) in patients with JSLE manifestations of pulmonary disease. Objectives To verify a subclinical pulmonary disease in juvenile onset SLE using laboratory parameters, pulmonary functions tests and multislice CT Methods Twenty five patients with JSLE were studied. All fulfilled updated American College of Rheumatology criteria for SLE [3]. All patients had the disease at or before the age of 16 years. Assessment of disease activity was done through SLE Disease Activity Index (SLEDAI) [4]. PFTs were done using (ZAN 100 Flow Handy II) pulmonary function apparatus. PFTs were done according to Grippi and Tino [5]. They included forced expiratory volume in 1 s as FEV1 (liter/min) and forced expiratory flow rate FEV1% of predicted FEV1, forced vital capacity FVC, residual volume RV, total lung capacity TLC, FEF 25-75%, FEF50% and FEF75%, and diffusion capacity of carbon monoxide DLCO. Abnormal PFTs were categorized into restrictive, obstructive, mixed restrictive, and obstructive, and small airway disease. Laboratory and immunological parameters were done for ANA, anti-dsDNA, ENA including Sm, and La, U1-snRNP, C3, C4, RF, Acl and LA. Plain radiography for chest and multislice CT were done. Results Twelve patients (48%) had abnormal results of PFTs, 5 (20%) had an isolated (DLCO) impairment and 6 (24%) had a restrictive pattern. Neurological lupus was significantly associated with decreased FEF25-75% (P<0.04) and FEF75% (P<0.001) respectively. Radiography was normal. Abnormal PFTs were not significantly associated with other parameters. Multislice CT examination of JSLE patients demonstrated a pleural effusion and thickening in 16%, also ground glass opacities found in 16% suggesting early interstitial lung disease. Conclusions A mandatory performance of pulmonary function tests have to be done early in JSLE, without findings of pulmonary involvement. References Torre O, Harari S. Pleural and pulmonary involvement in SLE Presse Med 2011;40 1 Pt 2):e19–29. Diane L, Kamen MD, Strange C. Pulmonary Manifestations of SLE Clin Chest Med 2010;31:479–88.2004; 43:587–91. Hochberg MC. Diagnostic and therapeutic criteria committee of the American college of rheumatology updating the American college of rheumatology revised criteria for the classification of SLE. Arthritis Rheum 1997; 40:1725. Bombardier C, Gladman DD, Urowitz MB, Caron D, Chang CH. Derivation of the SLEDAIA disease activity index for lupus patients. The committee on prognosis studies in SLE. ArthritisRheum 1992;35:630–40. Grippi MA, Tino G. Pulmonary function testing. In: Fishman AP, Elias JA, Fishman JA, Grippi MA, Senior RM, Pack AI, editors. Fishman’s pulmonary diseases and disorders...
Article informationBackground: Interstitial lung disease [ILD] is one of the most prevalent respiratory symptoms of connective tissue diseases [CTDs], leading to significant morbidity and death. Nailfold capillaroscopy [NFC] is the best tool for analysing microvascular disorders in CTDs since capillaroscopic alterations in the nailfold are well documented in several CTDs. The Aim of the work: To assess the value of Nailfold capillaroscopy examination in patients with ILD. Patients and methods: This study involved 100 patients aged between 18 and 90 years diagnosed with ILD divided into three groups; group [I] involved 30 patients with rheumatoid arthritis [RA] associated with ILD, group [II] involved 30 patients with systemic sclerosis [SSc] associated with ILD, and group [III] included 40 patients with idiopathic ILD. In all patients, NFC was performed with clinical and laboratory assessment. Results: According to nailfold capillaroscopic findings, patients with normal nailfold capillaroscopy were found only in idiopathic ILD. CTD-ILD group has a significantly lower mean capillary density than idiopathic ILD [p= <0.05]. Also, RA-ILD, IPF, and SSC-ILD subgroups had more severe abnormalities in capillaroscopy. The RA-ILD subgroup's capillary density was significantly lower than that of the idiopathic ILD only group. Furthermore, we found that [SSc-ILD] group had the lowest significant capillary number [ranged from 4-9/mm] & the highest significant capillary width [ranged from 29-76/μm] and the shortest significant capillary length [ranged from 75-256/μm] in comparison to the other groups. Conclusion:We can conclude that SSc-ILD patients had more severe nailfoldcapillaroscopic abnormalities than both RA-ILD and idiopathic ILD groups.Assessment of Nail should be considered during ILD examination for early detection of Nailfold abnormality to improve prognosis of the disease.
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