Subject(s)
Collagen Diseases , Humans , Collagen Diseases/chemically induced , Collagen Diseases/drug therapy , Collagen Diseases/pathology , Female , Glucocorticoids/administration & dosage , Glucocorticoids/adverse effects , Administration, Cutaneous , Male , Middle Aged , Adrenal Cortex Hormones/administration & dosage , Adrenal Cortex Hormones/adverse effects , Treatment OutcomeABSTRACT
Background: Acquired reactive perforating collagenosis (ARPC) poses a clinical challenge with an unclear pathogenesis. This disease has been frequently proven resistant to immunosuppressive treatments, significantly affecting the quality of life of patients. In this report, we highlight the efficacy of baricitinib as a viable option for maintenance therapy in ARPC. Case summary: An 81-year-old woman presented to our hospital with recurrent pruritus and cup-like ulcerated lesions on her trunk and limbs persisting for 1 year. She exhibited limited response to oral antihistamines and topical steroids. Past medical history revealed a prolonged history of coronary heart disease and type 2 diabetes spanning several years to decades. Histopathological examination revealed cup-shaped depressions filled with necrotic inflammatory debris. In the dermis, a mixed inflammatory infiltrate composed of lymphocytes and histiocytes was observed. Van Gieson staining indicated the elimination of fibrous tissue extending from the dermis into the epidermis. Consequently, a diagnosis of ARPC was established. Due to the inadequate response to conventional treatments and the severe itching, we initiated baricitinib therapy for ARPC, resulting in gradual symptom improvement. Follow-up assessments showed no adverse reactions and normal laboratory findings. Conclusion: The case report suggests that baricitinib might offer significant therapeutic benefits for ARPC.
Subject(s)
Azetidines , Collagen Diseases , Purines , Pyrazoles , Sulfonamides , Humans , Female , Azetidines/therapeutic use , Azetidines/adverse effects , Sulfonamides/therapeutic use , Pyrazoles/therapeutic use , Pyrazoles/adverse effects , Aged, 80 and over , Purines/therapeutic use , Purines/adverse effects , Collagen Diseases/drug therapy , Treatment Outcome , Skin/pathology , Skin/drug effectsABSTRACT
Immunosuppressive agents including steroids are generally given to patients with collagen disease or organ transplant recipients. Cardiovascular surgery for these patients can potentially associate with increased rate of postoperative infection or wound healing complications. Here, some key points for perioperative management in patients under immunosuppressive therapy are reviewed. Before an elective surgery, steroids need to be tapered down as much as possible, because even small amount of steroid can lead to adverse postoperative outcomes. Withholding Biologic disease-modifying antirheumatic drugs (bDMARDs) and Janus kinase (JAK) inhibitors is recommended for stable collagen disease patients. Current guidelines for perioperative management of antirheumatic medication are summarized in Table 1. Perioperative Glucocorticoid management is also required for patients exposed to steroid therapy. Intra-and postoperative steroid cover regimen is shown in Table 2. On the other hand, immunosuppressive therapy should not be discontinued for those after organ transplant and patients with severely active collagen disease. Our experience of kidney transplant recipients who underwent cardiovascular surgery is shown in Table 3. Close monitoring of blood Tacrolimus level is highly important, because it tends to fluctuate after operation and high Tacrolimus level possibly leads to deterioration in renal function. In conclusion, careful perioperative management in cooperation with transplant surgeons and rheumatologists is vital in this clinical setting.
Subject(s)
Cardiovascular Surgical Procedures , Immunosuppression Therapy , Immunosuppressive Agents , Humans , Collagen Diseases/drug therapy , Glucocorticoids/administration & dosage , Glucocorticoids/adverse effects , Immunosuppression Therapy/adverse effects , Immunosuppressive Agents/administration & dosage , Immunosuppressive Agents/adverse effects , Immunosuppressive Agents/therapeutic use , Kidney Transplantation , Tacrolimus/adverse effectsABSTRACT
BACKGROUND: Radiosynoviorthesis (RSO) is a nuclear medical local treatment modality for inflammatory joint diseases. It is indicated in patients with rheumatoid arthritis (RA) in joints with persistent synovitis despite adequate pharmacotherapy. Arthritis of the elbow joint occurs in up to 2/3 of patients with RA. Intra-articular radiotherapy using the beta emitter [186Re] rhenium sulfide leads to sclerosis of the inflamed synovial membrane with subsequent pain alleviation. The clinical efficacy in cubital arthritis, however, has so far only been described in small monocentric studies. OBJECTIVE: The degree of pain alleviation by RSO was analyzed in patients with rheumatoid cubital arthritis, treated in several nuclear medical practices specialized in RSO. MATERIAL AND METHODS: The subjective pain intensity before and after RSO was documented in a total of 107 patients with rheumatic cubital arthritis using a 10-step numeric rating scale (NRS). A difference of ≥â¯-2 is rated as a significant improvement. Follow-up examinations were done after a mean interval of 14 months after RSO (at least 3 months, maximum 50 months). RESULTS: The mean NRS value was 7.3⯱ 2.1 before RSO and 2.8⯱ 2.2 after RSO. A significant pain alleviation was seen in 78.5% of all patients treated. The subgroup analysis also showed a significant improvement in the pain symptoms in all groups depending on the time interval between the RSO and the control examination. A significant pain progression was not observed. The degree of pain relief was independent of the time of follow-up. CONCLUSION: Using RSO for local treatment of rheumatoid cubital arthritis leads to a significant and long-lasting pain relief in more than ¾ of the treated patients.
Subject(s)
Arthritis, Rheumatoid , Collagen Diseases , Elbow Joint , Rheumatic Diseases , Synovitis , Humans , Radioisotopes/adverse effects , Elbow , Synovitis/diagnosis , Synovitis/radiotherapy , Rheumatic Diseases/drug therapy , Arthritis, Rheumatoid/complications , Arthritis, Rheumatoid/diagnosis , Arthritis, Rheumatoid/radiotherapy , Collagen Diseases/drug therapy , Treatment Outcome , Pain/diagnosis , Pain/etiology , Pain/radiotherapyABSTRACT
Rheumatic diseases have been reported to sometimes involve the pituitary gland. This study aims to characterize the clinical features and outcomes of patients with rheumatic disease-associated hypophysitis. We used the electronic medical record system in our hospital to identify nine patients with pituitary involvement in rheumatoid disease. We summarized the clinical characteristics, radiographic findings, treatments, and clinical outcomes of the 9 patients. We also performed a systematic literature review of systemic lupus erythematosus (SLE) cases with pituitary involvement published in PubMed and Wanfang databases from 1995 to 2021, and eight patients with complete information were selected. In the nine-patient cohort, the median age was 54 years, and the spectrum of rheumatic diseases included immunoglobulin G4-related disease (IgG4RD) (4/9), SLE (2/9), vasculitis (2/9), and Sjögren syndrome (SS) (1/9). All patients had pituitary abnormalities on radiological assessment, 6 developed diabetes insipidus (DI), and 8 presented with anterior pituitary hormone deficiencies in the disease duration. All the patients had multisystem involvement. As compared to hypophysitis with IgG4RD (IgG4-H), the age at onset of hypophysitis with SLE (SLE-H) patients was younger [(30.4 ± 16.4) years vs. (56.0 ± 0.8) years] and the disease duration was shorter [(14.0 ± 17.5) months vs. (71.0 ± 60.9) months] (P < .05). All patients were managed with glucocorticoids (GC) in combination with another immunosuppressant, and the majority of patients improved within 4 months. Six patients achieved disease remission while four required at least one hormone replacement therapy. Hypophysitis is a rare complication secondary to a variety of various rheumatic diseases that can occur at any stage. GC combined with additional immunosuppressants could improve patients' symptoms; however some patients also required long-term hormone replacement therapy in pituitary disorders.
Subject(s)
Autoimmune Hypophysitis , Collagen Diseases , Hypophysitis , Hypopituitarism , Lupus Erythematosus, Systemic , Pituitary Diseases , Rheumatic Diseases , Humans , Middle Aged , Adolescent , Young Adult , Adult , Hypophysitis/complications , Pituitary Diseases/complications , Pituitary Diseases/diagnosis , Hypopituitarism/etiology , Pituitary Gland/diagnostic imaging , Glucocorticoids/therapeutic use , Rheumatic Diseases/complications , Rheumatic Diseases/drug therapy , Immunosuppressive Agents/therapeutic use , Collagen Diseases/drug therapy , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/drug therapy , Autoimmune Hypophysitis/complications , Autoimmune Hypophysitis/diagnosis , Autoimmune Hypophysitis/drug therapyABSTRACT
BACKGROUND/AIM: To investigate factors associated with increased bone mineral density (BMD) of the neck of femur in rheumatoid arthritis or collagen diseases receiving denosumab, focusing on body composition calculated by bioelectrical impedance analysis (n=90, 78 females). PATIENTS AND METHODS: We defined Δfemur as BMD (12 months minus baseline), using dual-energy X-ray absorptiometry after denosumab therapy. Factors associated with Δfemur were retrospectively investigated. RESULTS: Low skeletal muscle index (SMI) was observed in 6 males and 32 females. There was a significant difference in phase angle (PhA) of the left leg (LL) between the Δfemur ≥0 (n=70) and Δfemur <0 (n=20) groups (p=0.040) but not in SMI (p=0.310). Multiple regression analysis indicated that PhA of LL was significantly related to Δfemur (p=0.0398). CONCLUSION: PhA appears to be a clinically significant indicator of improvement of Δfemur in patients receiving denosumab.
Subject(s)
Arthritis, Rheumatoid , Collagen Diseases , Arthritis, Rheumatoid/drug therapy , Body Composition/physiology , Bone Density , Collagen Diseases/complications , Collagen Diseases/drug therapy , Denosumab/adverse effects , Female , Femur Neck/diagnostic imaging , Humans , Male , Retrospective StudiesSubject(s)
Collagen Diseases , Dipeptidyl-Peptidase IV Inhibitors , Skin Diseases , Collagen Diseases/drug therapy , Dipeptidyl-Peptidase IV Inhibitors/adverse effects , Dipeptidyl-Peptidases and Tripeptidyl-Peptidases , Humans , Hypoglycemic Agents , Skin Diseases/drug therapy , Skin Diseases/etiologyABSTRACT
The management of acquired reactive perforating collagenosis (ARPC) is challenging. Here, we shared two cases of ARPC combined with elderly atopic dermatitis (AD) that did not respond well to conventional treatment but responded well to the monotherapy of dupilumab, which suggests that dupilumab may be an alternative option for the treatment.
Subject(s)
Collagen Diseases , Dermatitis, Atopic , Skin Diseases , Aged , Antibodies, Monoclonal, Humanized/therapeutic use , Collagen Diseases/complications , Collagen Diseases/drug therapy , Dermatitis, Atopic/complications , Dermatitis, Atopic/drug therapy , HumansABSTRACT
BACKGROUND: Acquired reactive perforating collagenosis (ARPC) is a rare form of transepithelial elimination in which altered collagen is extruded through the epidermis. CASE PRESENTATION: A 23-year-old male presented with cup-like ulcerated lesions on his limbs since 3 months. A series of serological and immunological tests showed no abnormalities. A diagnosis of ARPC was based on skin biopsy findings. The patient was cured using treatment with itraconazole for 8 weeks, in the absence of a fungal infection. CONCLUSIONS: The anti-inflammatory and anti-angiogenic effects of itraconazole can have good therapeutic benefits for ARPC.
Subject(s)
Antifungal Agents/therapeutic use , Collagen Diseases/drug therapy , Itraconazole/therapeutic use , Skin Diseases/drug therapy , Adult , Collagen Diseases/pathology , Humans , Male , Prognosis , Skin Diseases/pathology , Young AdultABSTRACT
No disponible
Subject(s)
Humans , Male , Aged, 80 and over , Sorafenib/adverse effects , Drug Eruptions/etiology , Collagen Diseases/chemically induced , Skin Diseases/chemically induced , Protein Kinase Inhibitors/administration & dosage , Protein Kinase Inhibitors/adverse effects , Biopsy , Skin/drug effects , Histamine Antagonists/administration & dosage , Collagen Diseases/diagnosis , Collagen Diseases/drug therapyABSTRACT
INTRODUCTION: Acquired reactive perforating collagenosis (ARPC) is a rare skin disorder, which is associated with various internal diseases and even malignant neoplasms. A comprehensive knowledge of the concomitant diseases in ARPC patients is helpful to decrease the misdiagnosis. Although the treatment of ARPC is challenging, systemic assessment of existing regimens is not available. PATIENT CONCERNS: A 50-year-old woman was admitted to the hospital due to cutaneous pruritus and papules all over the body. DIAGNOSIS: Physical examination showed various sized papules on the lower limbs, buttocks, back, chest, and upper arms with keratotic plugs in the center. Histopathology showed typical collagenous fiber perforation. The diagnosis of ARPC was made according to histopathology, onset age and typical skin lesions. Type 2 diabetes mellitus (T2DM), chronic renal failure (CRF), and hypothyroidism simultaneously presented in this patient. INTERVENTIONS: This patient was initially treated with topical corticosteroids and oral antihistamines for the skin lesion and pruritus. Medications for glucose control and recovery of renal and thyroid functions were also applied. On the second admission, the combined therapy of topical retinoic acid, Chinese medicinal herb-Qingpeng ointment, and Zinc oxide ointment was added. OUTCOMES: Papules and pruritus were improved significantly after the second hospitalization. CONCLUSION: We present a case of ARPC associated with T2DM, CRF, and hypothyroidism, which has rarely been described. There is no standardized treatment for ARPC. Co-administration of two or more agents for dermatologic interventions and treatment for associated diseases may help to improve skin symptoms.
Subject(s)
Collagen Diseases/diagnosis , Skin Diseases/diagnosis , Collagen Diseases/drug therapy , Collagen Diseases/etiology , Collagen Diseases/pathology , Dermatologic Agents/therapeutic use , Diabetes Mellitus, Type 2/complications , Female , Humans , Kidney Failure, Chronic/complications , Middle Aged , Skin/pathology , Skin Diseases/drug therapy , Skin Diseases/etiology , Skin Diseases/pathologyABSTRACT
No disponible
Subject(s)
Humans , Male , Aged , Collagen Diseases/etiology , Kidney Failure, Chronic/complications , Skin Diseases/etiology , Antigens, Neoplasm/metabolism , Cetirizine/therapeutic use , Collagen/metabolism , Collagen Diseases/drug therapy , Collagen Diseases/physiopathology , Diabetes Mellitus, Type 2/complications , Fluocinolone Acetonide/therapeutic use , Hypertension/complications , Hypothyroidism/complications , Kidney Failure, Chronic/therapy , Mitogen-Activated Protein Kinases/metabolism , Peritoneal Dialysis , Skin Diseases/drug therapy , Skin Diseases/physiopathologySubject(s)
Collagen Diseases/etiology , Kidney Failure, Chronic/complications , Skin Diseases/etiology , Aged , Antigens, Neoplasm/metabolism , Cetirizine/therapeutic use , Collagen/metabolism , Collagen Diseases/drug therapy , Collagen Diseases/physiopathology , Diabetes Mellitus, Type 2/complications , Fluocinolone Acetonide/therapeutic use , Humans , Hypertension/complications , Hypothyroidism/complications , Kidney Failure, Chronic/therapy , Male , Mitogen-Activated Protein Kinases/metabolism , Peritoneal Dialysis , Skin Diseases/drug therapy , Skin Diseases/physiopathologySubject(s)
Benzoyl Peroxide/administration & dosage , Collagen Diseases/drug therapy , Dermatologic Agents/administration & dosage , Skin Diseases/drug therapy , Administration, Oral , Administration, Topical , Aged , Collagen Diseases/pathology , Glucocorticoids/administration & dosage , Histamine Antagonists/administration & dosage , Humans , Male , Pruritus/drug therapy , Pruritus/etiology , Skin Diseases/pathologyABSTRACT
Basement membranes (BMs) are specialised extracellular matrix (ECM) structures and collagens are a key component required for BM function. While collagen IV is the major BM collagen, collagens VI, VII, XV, XVII and XVIII are also present. Mutations in these collagens cause rare multi-systemic diseases but these collagens have also been associated with major common diseases including stroke. Developing treatments for these conditions will require a collective effort to increase our fundamental understanding of the biology of these collagens and the mechanisms by which mutations therein cause disease. Novel insights into pathomolecular disease mechanisms and cellular responses to these mutations has been exploited to develop proof-of-concept treatment strategies in animal models. Combined, these studies have also highlighted the complexity of the disease mechanisms and the need to obtain a more complete understanding of these mechanisms. The identification of pathomolecular mechanisms of collagen mutations shared between different disorders represent an attractive prospect for treatments that may be effective across phenotypically distinct disorders.