Immunoglobulin G4-Related Lesions in Autoimmune Diseases: Unusual Presentations at Atypical Sites-A Tale of 2 Cases with Literature Review.

Immunoglobulin G4-related disease (IgG4-RD) coexisting with clinically apparent autoimmune diseases, such as rheumatoid arthritis (RA) or antiphospholipid syndrome (APS), is a rarely documented combination in the scientific literature. In this case-based review, we present 2 intriguing cases with preexisting autoimmune diseases, namely, RA and primary APS, who exhibited coexistent IgG4- related lesions at unusual sites. The first case pertains to a patient with known RA who presented with an encasing mass in the esophagus leading to stricture, with histopathological diagnosis of IgG4-RD.The second patient, diagnosed with primary APS, experienced breathlessness, and imaging revealed a right atrial mass. Histopathological examination of the mass confirmed IgG4-RD. Notably, both patients demonstrated significant clinical improvement upon initiation of steroid therapy. Rheumatoid arthritis patients commonly exhibit elevated levels of IgG4 in their sera; however, RA with coexisting IgG4-RD is rarely reported in the literature. Similarly, APS with IgG4-related lesions is exceedingly rare. Although there are few case reports and series on esophageal and cardiac IgG4-RD, the occurrence of such unusual location of IgG4-related lesions in the context of known autoimmunity is presented here for the first time.

A Case of SAPHO Syndrome Complicated by Uveitis with Good Response to Both TNF Inhibitor and JAKinib.

Introduction: SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome is a rare autoinflammatory condition describing the constellation of inflammatory skin, bone, and joint manifestations which result in diagnostic difficulty and therapeutic challenge. Case: Here, we present a case of a young male diagnosed with SAPHO syndrome with osteoarticular and cutaneous involvement from an early age in his life. He suffered diagnostic challenges for a long time and was hence inadequately treated. He had minimal response to conventional DMARDs but showed excellent response to TNF inhibitor (adalimumab). Later, he defaulted treatment and presented with acute anterior uveitis which was also dramatically improved with adalimumab and tofacitinib although financial constraint was always an issue for the patient. Conclusion: The uniqueness of this case was that the patient had a multiorgan involvement including osteoarticular system, skin, and eye. Both TNFi (adalimumab) and JAKinib (tofacitinib) had a good response to all organs with a net improvement in the quality of life of this patient.

Postpartum Myasthenic Crisis in a Patient with SLE: A Case Report and Literature Review.

Background: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease characterized by the presence of numerous autoantibodies while Myasthenia Gravis (MG) is an organ-specific autoimmune disease. The coexistence of both diseases is rarely reported in the literature. Case presentation: We report a case of a 29-year-old female SLE patient with chief manifestations of nephritis, inflammatory polyarthritis and cytopenia presented with postpartum shortness of breath and dysphagia requiring emergency intubation and difficulty in weaning. Later she developed chronic respiratory acidosis with bilateral ptosis. Her diagnosis of myasthenia was confirmed with a positive neostigmine test and nAChR antibodies. She was given 5 cycles of PLEX and pyridostigmine with significant improvement of symptoms and extubated safely. Conclusion: It is one of the rare case reports of SLE preceding MG with significant improvement by anticholinergic therapy.

Treatment outcomes in refractory lupus nephritis: Data from an observational study.

OBJECTIVES: Despite current advances in treatment, refractory lupus nephritis (RLN) continues to pose a challenge. The present paper studies the clinical profile and treatment outcomes in patients with RLN. METHODS: This observational, bidirectional study enrolled consecutive lupus nephritis (LN) patients from August 2018 to January 2019, who either failed to improve within three months, did not achieve partial renal response (PR) at six months, or did not achieve complete renal response (CR) after two years of treatment. Patients were followed every three months; treatment details and outcomes [CR, PR, no renal response (NR)], doubling serum creatinine, and death were recorded. Group comparisons were made using ANOVA and chi-square test. Factors affecting renal response were studied using linear regression. RESULTS: Forty-five of forty-eight enrolled patients completed at least nine months of follow-up and were included in outcome analysis. The median (IQR) SLE duration was three years (2-6 years). The majority of patients (n = 25) had proliferative LN (ISN/RPS class III/IV), with nine patients having pure membranous LN (class V). The mean activity and chronicity indices were 8 and 0. Over a median (IQR) follow-up period of 15 (12-27) months, 28 had CR, 9 had PR, and 8 showed no response to a switch in an immunosuppressive (IS) agent. Repeat renal biopsy (n = 8) with a mean (±SD) biopsy interval of 2 (±1) years showed histological class transformation in more than half of the patients. There was no significant difference in treatment outcome and time to attain response based on individual IS agent or sequence of IS agents used. None of the variables (duration of SLE or nephritis, baseline SLEDAI, leukopenia, hypertension, elevated anti-dsDNA, low complements, serum albumin, 24-hour urinary protein, biopsy class) predicted renal response on univariate analysis. No patient had a doubling of serum creatinine or progression to end-stage renal disease. There were three deaths, all related to infection. CONCLUSION: A change in immunosuppression produces response in most RLN patients while a fifth of them showed no response to therapy. No predictor of renal response was identified. Histologic class switch was frequent. Renal function did not decline over a year of follow-up.

A review of COVID-19 convalescent plasma use in COVID-19 with focus on proof of efficacy.

Convalescent plasma (CP) therapy is rapidly becoming an established consideration in the treatment of COVID-19 patients though there is a need to critically review this area for proof of efficacy. Neutralizing antibodies (NAb) present in CP generated in response to SARS-CoV-2 infection directed against the receptor-binding domain (RBD) of the spike protein are considered to play main role in viral clearance. CP infusion may also help in the modulation of immune response by its immunomodulatory effect. The FDA allows for administration of CP to COVID-19 patients. The present published literature in COVID-19 is limited to case series and randomised controlled trial where plasma therapy was used in moderate, severe and critically ill patients. Though multiple uncertainties exist regarding to its efficacy, appropriate donor selection and NAb titres, the efficacy data of CP use inCOVID-19 is limited having shown hope with early and severe to critically ill COVID-19 patients.

Bronchiolitis obliterans organizing pneumonia as the pulmonary manifestation of lupus: A review of three cases.

OBJECTIVE: Bronchiolitis obliterans organizing pneumonia (BOOP) is a clinico-patho-radiological diagnosis which rarely presents as a pulmonary manifestation of lupus. In this concise report, organizing pneumonia was found as the sole pulmonary manifestation of SLE in different age groups. METHOD: All three patients diagnosed with SLE according to SLICC 2012 classification criteria, were admitted in rheumatology ward of NIMS hospital, Hyderabad, India from May to November, 2018. Their diagnosis of BOOP was either biopsy proven or imaging guided. Review of literature was done with MeSH terms (SLE, BOOP) in PubMed and approximately 10 articles were reviewed including latest of 2019 published in Scientific Reports. RESULT: There were three patients - one juvenile lupus and two adults. Two patients were male and one female. All three patients had SLE with high disease activity. They all had organising pneumonia as pulmonary manifestation with other organ involvement. Juvenile patient had a fatal outcome while the others had a good recovery with steroid and immunosuppressive. CONCLUSION: BOOP is a rare pulmonary manifestation in lupus. It can be diagnosed early with more precision using computerised tomography of lung without waiting for biopsy report. This will result in a better prognosis by rapid initiation of corticosteroid and immunosuppressive treatment.

Diabetic myonecrosis: an unusual mimicker of idiopathic inflammatory myositis.

Diabetic myonecrosis or diabetic muscle infarction (DMI), is a very rare and under-recognised complication of poorly controlled long-standing diabetes mellitus. We report a case of a 59-year-old male, who had diabetes for ten years. He presented with bilateral thigh pain of insidious onset for three months and difficulty in walking, with a similar episode in his right thigh in 2015. Creatine phosphokinase (CPK) was one and half times the normal upper limit. Magnetic resonance imaging (MRI) of his thighs showed symmetrical bulky muscles with hyperintensities on T2-weighted and short tau inversion recovery (STIR) images, supporting a clinical diagnosis of idiopathic inflammatory myositis (IIM). However, a review of histopathology slides of a muscle biopsy from the right vastus lateralis performed in 2015 showed muscle fibre ischaemic necrosis suggestive of muscle infarction. Thus a diagnosis of recurrent diabetic myonecrosis was made and the patient was treated with bed rest, opioids and aspirin with gradual recovery.

Methotrexate achieves major cDAPSA response, and improvement in dactylitis and functional status in psoriatic arthritis.

OBJECTIVE: Despite the widespread clinical use of MTX in PsA, data from published randomized controlled studies suggest limited efficacy. The objective of the present study was to document the efficacy of MTX. METHODS: This was an open-label, prospective study of patients satisfying the ClASsification criteria for Psoriatic ARthritis study (CASPAR) criteria for PsA who received MTX in doses of ⩾15 mg/week throughout the follow-up period of 9 months. Disease activity was assessed across various domains by tender and swollen joint count, physician and patient global assessment, DAS-28 ESR, Clinical Disease Activity Index for PsA (cDAPSA), Leeds Dactylitis Instrument basic, Leeds Enthesitis Index (LEI), Psoriasis Area and Severity Index (PASI), Minimal Disease Activity and HAQ (CRD Pune version) at baseline and at 3, 6 and 9 months of follow-up. Response to therapy was assessed by EULAR DAS28 ESR, Disease Activity Index for PsA (cDAPSA) response, HAQ response and PASI75. MTX dose escalation and the use of combination DMARDS were dictated by disease activity. RESULTS: A total of 73 patients were included, with mean (s.d.) age 44 (9.7) years. The mean (s.d.) dose of MTX used was 17.5 (3.8) mg/week. Seven patients received additional DMARDS (LEF/SSZ). At the end of 9 months, significant improvement (P < 0.05) was noted in the tender joint count, swollen joint count, global activity, DAS-28ESR, cDAPSA, Leeds Dactylitis Index basic, LEI, PASI and HAQ. Major cDAPSA response was achieved in 58.9% of patients. EULAR DAS28 moderate and good response was achieved in 74% and 6.8% of patients, respectively. Minimal Disease Activity was achieved in 63% of patients. A PASI75 response and HAQ response was achieved in 67.9% and 65.8% of patients, respectively. CONCLUSION: MTX initiated at ⩾15 mg/week with targeted escalation resulted in significant improvement in the skin, joint, dactylitis, enthesitis and functional domains of PsA.