A Study of Mucocutaneous Manifestations in Autoimmune Connective Tissue Disorders at Tertiary Care Centre

Abstract

BACKGROUND Our aim was to study the clinical and immunological profile of patients with newly detected connective tissue disease presented to a tertiary care centre. MATERIALS AND METHODS The study involved 51 patients with newly-detected Systemic Lupus Erythematosus (SLE) (fulfilling the revise SLICC criteria for SLE) and Systemic Sclerosis (SS), Mixed Connective Tissue Disease (MCTD), etc. attending Sir. T. Hospital, Bhavnagar, between January 2013 and December 2016. All patients were assessed for clinical features and immunological profile. RESULTS Out of the 51 patients, 30 having SLE, 10 having SS, 9 with MCTD, 1 with dermatomyositis and 1 with Rowell's syndrome. Among them, 47 were females and 4 were males. The mean age at presentation was between 15-25 years. The LE-specific skin lesions were noted as malar rash in 25 patients (83%), subacute and acute lupus rashes (80%) and discoid rash (13%). Among LE-nonspecific lesions, non-scarring alopecia was most common followed by oral ulcers, Raynaud's phenomenon, joint pain, scarring alopecia, erythema multiforme, livedo reticularis, vasculitic lesions, urticaria and calcinosis cutis were seen. In MCTD, muscle weakness was common finding. In systemic sclerosis, hide-bound skin and decreases mouth opening were seen in all cases and Raynaud's phenomenon, joint pain, hair loss, calcinosis cutis and respiratory system involvement were other features. Serum ANA was positive in 76% while negative in 3.8% of individuals. The most common pattern observed in ANA profile was speckled (56%) followed by homogenous (32%) and nucleolar (28%). CONCLUSION There is diversity in clinical presentation of autoimmune connective tissue disease with regards to their genetic and environmental backgrounds. Cutaneous features are utmost important having diagnostic and prognostic value as well.

An observational cross-sectional study of varied clinical manifestations of connective tissue disorders and their association with antinuclear antibodies in a tertiary care center

“…Anti-ENA profile positivity of certain antibodies like Sm, dsDNA, U1-RNP, and Scl-70 are markers of underlying organ damage and clinically relevant disease manifestations. [5815] SLE patients with renal involvement showed the presence of anti-Sm and dsDNA antibodies, which was in accordance with previous studies. [1516] Anti-Sm antibodies are thought to be predictors of silent lupus nephritis.…”

“…The demographic and clinical profiles of our patients were comparable to other studies. [45] Akin to the original description of the disease, our study also showed that CTDs primarily affect middle-aged people. [45] The onset of SLE in childhood is associated with an increased severity of manifestation of the disease, higher frequency of organ damage, and reduced survival rates.…”

“…[45] Akin to the original description of the disease, our study also showed that CTDs primarily affect middle-aged people. [45] The onset of SLE in childhood is associated with an increased severity of manifestation of the disease, higher frequency of organ damage, and reduced survival rates. [6] We encountered a rare case of Rowell syndrome (RS) with lupus erythematosus and erythema multiforme like lesions with antibodies to SS-A.…”

“…The spectrum and frequencies of cutaneous manifestations observed were in accordance with other studies. [51213]…”