Glomerulopathy in patients with dermatomyositis in early active disease: clinical, pathological and capillaroscopic manifestations, and response to treatment.

Introduction: Idiopathic inflammatory myopathies (IIMs) are a group of systemic connective tissue diseases that present with muscular and extra-muscular manifestations. There are few reports on kidney involvement, especially in dermatomyositis (DM) patients. We evaluated the clinical, laboratory, capillaroscopy, and kidney pathology of patients with DM, who presented with proteinuria during the first year, and followed them for response to treatment. Material and methods: We evaluated 205 patients with proximal muscle weakness or high muscle enzymes, who referred to the nailfold capillaroscopy clinic from April 2010 to October 2021. Seventy-four patients fulfilled the New 2017 EULAR/ACR Classification Criteria for adult and juvenile IM with probability of ≥ 90% for DM with duration of ≤ 12 months and proteinuria > 350 mg/24 hours. All manifestations of patients with glomerulopathy and their kidney biopsies were reviewed, and they were followed for their treatment response. Results: From 74 patients with DM, 52 female and 22 male, median age 37 (19-65) years, and disease duration of median 4.5 (1-12) months, 2 (2.7%) patients (25- and 28-year-old male) had proteinuria. Their kidney biopsy showed mesangioproliferative glomerulonephritis (GN). There was no case of acute or chronic kidney damage or rhabdomyolysis. Both had high disease activity, high erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), abnormal capillaroscopy, and high anti-Ro positivity with good early response of their kidney function, muscle weakness, and laboratory tests after immunosuppressive treatment for 3-6 months. One patient had capillaroscopy follow-up, and all abnormalities were resolved in 8 fingers. One patient, due to poor follow-up, after 8 months had recurrence of his disease. Conclusions: We found mesangioproliferative GN as a rare extra-muscular manifestation in patients with DM in the active and early phase of the disease. Full immunosuppressive treatment showed early complete recovery in these patients.

Mechanic hands: clinical and capillaroscopy manifestations of patients with connective tissue diseases presented with and without mechanic hands.

OBJECTIVES: The condition known as 'Mechanic's Hands' is a thickened, hyperkeratotic eruption, which is bilaterally symmetric along the fingers, and often occurs in patients with some connective tissue diseases. Nail fold capillaroscopy is a non-invasive technique for evaluation of connective tissue diseases. We evaluated the prevalence of mechanic hands in patients with connective tissue diseases and compared the clinical manifestations and capillaroscopic changes in the patients with and without mechanic hands. METHODS: The clinical manifestations and capillaroscopy of 576 patients with scleroderma, dermatomyositis, systemic lupus erythematosus, Sjogren's syndrome, undifferentiated and mixed connective tissue diseases were evaluated and compared in patients with and without mechanic hands. RESULTS: A total of 576 patients were enrolled. Mechanic hands were observed in 17.2% of patients: 50% of mixed connective tissue disease, 35% of dermatomyositis, 15.4% of scleroderma, 14.9% of undifferentiated connective tissue disease, 14.3% of Sjogren's syndrome, and no patient with SLE. Among them, 80.8% had abnormal capillaroscopic findings. In dermatomyositis patients, Raynaud's phenomenon, anti-Jo-1 positivity, and some capillaroscopy findings were detected more frequently in patients with mechanic hand. In scleroderma, positive Scl70 and capillary loss were observed more frequently in patients without mechanic hands. CONCLUSIONS: Mechanic hands can be a presenting sign of some systemic connective tissue diseases. Probably, finding this sign on examination, especially together with Raynaud's phenomenon or abnormal capillaroscopy, can be helpful in the early diagnosis of the connective tissue diseases and can be used as a predictive and prognostic tool in future studies.

Conjunctivitis as a manifestation of Wegener's Granulomatosis.

PURPOSE: To report a case of prolonged conjunctivitis as the manifestation of Granulomatosis with polyangiitis (GPA). METHODS: A 37-year-old man presented with prolonged conjunctivitis which had persisted for one month. He was taking medication for his conjunctivitis without any response. A slit-lamp examination revealed conjunctivitis and scleritis in the right eye. Conjunctivitis, 360-degree peripheral corneal thinning, corneal perforation, and scleritis were seen in the left eye. RESULTS: Emergency penetrating keratoplasty was performed to treat the patient's corneal perforation. After a consultation with the Internal Medicine Department, the patient was suspected of having GPA with positive cytoplasmic anti-neutrophil cytoplasmic antibodies (C-ANCA). Functional endoscopic sinus surgery was performed to treat right maxillary sinusitis, and a biopsy of the maxillary sinus mucosa was obtained. The pathology report showed granuloma and vasculitis with severe acute and chronic inflammation and few eosinophils; thus, the diagnosis was confirmed. CONCLUSION: Because prolonged conjunctivitis occurs only rarely in association with systemic disease, ophthalmologists should be aware of this potential, particularly in patients with GPA.

Three atypical manifestations of granulomatosis with polyangiitis: lateral medullary syndrome, anterior cheek mass and melting scleritis of eye.

Granulomatosis with polyangiitis (GPA, formerly Wegener granulomatosis) is a vasculitis with various organ involvement. There have been a few cases of CNS stroke and rare cases of lateral medullary infarction (LMI) as a manifestation of GPA. Also there have been reports of sinuses, nose and laryngeal masses mistakenly referred as carcinomas and subsequently GPA was diagnosed in their pathological reports. Another severe fulminant manifestation can be necrotizing scleritis leading to perforation of sclera. Therefore, here we present some rare and fulminant manifestations of GPA in 3 separate cases for further emphasis of the unusual manifestations of GPA that should always be kept in mind.

Diabetic muscle infarction and diabetic dermopathy two manifestations of uncontrolled prolong diabetes mellitus presenting with severe leg pain and leg skin lesions.

Diabetic muscular infarction (DMI) is a rare manifestation which can be seen in patients with long-standing diabetes mellitus. Patients usually come with painful swelling of an involved muscle in one extremity. MRI and biopsy histology can help diagnose this condition. Diabetic dermopathy is another manifestation of patients with diabetes.We present a patient with uncontrolled diabetes type 2 presented with pain, swelling, and a palpable tender mass in one leg along with new skin lesions. Biopsy of the skin lesion and T2-weighted MRI of the leg helped differentiate DMI and dermopathy.

Infliximab for patients with neuro-Behcet's disease: case series and literature review.

This study aims to report the therapeutic effects of anti-tumor necrotic factor antibody, infliximab, for treatment of neuro-Behcet's disease (NBD) and to review the literature. We described four patients (all male, median age 40 years old) who fulfilled the International Study Group criteria for Behcet's disease (BD) and presented with neurological complication. Demographic and clinical characteristics of the patients, dose, therapeutic effects, and adverse drug reaction (ADR) of infliximab were reported. Two patients had secondary progressive, one relapsing progressive, and one primary progressive course (median duration of BD and NBD 11 and 2 years, respectively). Two patients each received infliximab with 3 and 5 mg/kg infusions, respectively. The patients received infliximab for median of 22 weeks. Clinical responses were unsatisfactory for two patients on 3 mg/kg regimen; and good in two patients on 5 mg/kg and monthly intravenous 500-1,000 mg cyclophosphamide. Varicella zoster infection was seen as a major ADR in one patient. Our results with infliximab were not as promising as the previous reports. Infliximab, 5 mg/kg per dose with adjuvant immunosuppressive therapy, is probably more effective than other regimens.