Clinically amyopathic dermatomyositis is a subset of dermatomyositis that does not have any clinical evidence of muscle inflammation. Hence, it frequently poses a diagnostic challenge to the clinician. Here, we present a middle-aged farmer who presented only with multiple non-healing ulcers and was eventually found to be having early interstitial lung disease. He was finally diagnosed with anti- melanoma differentiation-associated gene 5 dermatomyositis and was started on aggressive immunosuppressants.
Aims: Infection is a major trigger for both Lupus flare and Macrophage activation syndrome. Scrub typhus infection associated with lupus flare or MAS has been very rarely reported. We report a case of scrub typhus-induced MAS along with a flare of disease activity in a neuropsychiatric lupus patient presenting after discontinuation of medications.
Presentation of Case: 13-year-old girl, a known case of neuropsychiatric lupus with lupus nephritis, on immunosuppressant for the last 1 year had stopped all medications for 3 months. She had presented with a fever and altered sensorium. Based on clinical and laboratory investigations, a diagnosis of scrub typhus-induced macrophage activation syndrome with a flare of lupus disease activity was made and the patient was treated with injection doxycycline and glucocorticoids. There was rapid clinical improvement with treatment.
Discussion: The case emphasizes the importance of identifying the exact trigger behind the deterioration of a patient with lupus and its therapeutic implications. An infection alone requires antibiotics only, flare without infection requires up titrating immunosuppressants but the presence of life-threatening complications like MAS along with infection requires prompt treatment of both facets simultaneously.
Conclusion: Scrub typhus infection should be ruled out apart from other common infections in any patient with lupus flare or MAS.
Blindness due to retinal vascular thrombosis is a dreadful complication of antiphospholipid syndrome (APS). The latter may be present in isolation (ie, primary) or may occur secondary to other connective tissue diseases like lupus. Here we present an adolescent girl with bilateral painless loss of vision as a result of central retinal vessel thrombosis due to secondary APS. Her condition was further complicated by the presence of autoimmune haemolytic anaemia and the development of macrophage activation syndrome while being evaluated for her blindness. Prompt treatment with glucocorticoids and anticoagulants could halt the devastating disease process.
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