|Year : 2022 | Volume
| Issue : 3 | Page : 731-733
Granulomatosis with polyangiitis affecting nervous system
Vivek Ramanandi, Kakkad Ashish, Anjan Desai
Department of Neuro Physiotherapy, SPB Physiotherapy College, Surat, Gujarat, India
|Date of Submission||31-Jan-2020|
|Date of Decision||19-Sep-2020|
|Date of Acceptance||27-Dec-2020|
|Date of Web Publication||2-Nov-2022|
Dr. Vivek Ramanandi
SPB Physiotherapy College, Surat, Gujarat
Source of Support: None, Conflict of Interest: None
Central nervous system involvement in granulomatosis with polyangiitis is quite rare condition. Involvement of multiple systems makes the management of condition difficult. Role of physiotherapy for such a rare condition is unexplored. In this case report, the disease and its medical and physiotherapy management are discussed in brief.
Keywords: Granulomatosis with polyangiitis, mononeuropathy, physiotherapy, quality of life, stroke
|How to cite this article:|
Ramanandi V, Ashish K, Desai A. Granulomatosis with polyangiitis affecting nervous system. J Datta Meghe Inst Med Sci Univ 2022;17:731-3
| Introduction|| |
Granulomatosis with polyangiitis (GPA) (i.e., Wegener's granulomatosis) (GPA) is a rare, multisystemic, life-threatening condition of unknown etiology having autoimmune inflammatory origin. The American College of Rheumatology defined GPA by the presence of two out of these four criteria: nephritic urinary sediment, abnormal chest finding, nasal or oral inflammation, and granulomatous inflammation on biopsy. It is an antineutrophil cytoplasmic antibody (ANCA)-associated small-vessel vasculitides. The diagnosis is based on the clinical criteria, including oral ulcers and an abnormal chest X-ray, which is supported by histopathological findings which reveal granulomatous inflammation and positive C-ANCA and PR3 in the laboratory studies. Common treatment regimen includes use of combination of corticosteroids and cyclophosphamide or other immunosuppressive agents.
Involvement of the peripheral nervous system (PNS) is relatively common as compared to the central nervous system (CNS). Manifestations in CNS are reported in <11% and are rare at disease onset of the disease., Here, a rare case of a patient with involvement of the PNS as well as CNS presented at the onset of the disease along with integumentary symptoms is reported. Based on this case and a review of the literature, the pathogenic mechanisms, clinical features, physiotherapy treatment, and outcomes are discussed.
| Case Report|| |
A 36-year-old female patient presented with chest infection before 4 years and was investigated for chest X-ray indicative of atelectasis, prominent vascular marking, cardiomegaly, and presence of fibrotic band. High-resolution computerized tomography scan was diagnostic of Wegener's granulomatosis. Since then, she had episodic relapses and remission of glomerulonephritis with pain and weakness in different body parts. A recent episode of right lower limb and left upper limb swelling, pain, and weakness along with darkening of skin was identified as a triad of mononeuritis multiplex, acute left middle cerebral artery stroke, and cutaneous rashes. The patient was under regular medical management under a rheumatologist and a physician. The patient was then referred to physiotherapy department for further management.
The patient was investigated by different laboratory tests periodically, and abnormalities such as reduced hemoglobin percentage (11 g%–11.9 g%); altered blood indices; increased white blood cells count (11,700 cumm–18800/cumm); elevated erythrocyte sedimentation rate (21 mm/h–70 mm/h); positive serum C-reactive protein (13.41 mg/L–26.2 mg/L); borderline random blood sugar level (126.23 units); presence of ANCA (p-ANCA/myeloperoxydase: negative, i.e., <5 AU/ML, c-ANCA/PR3: positive, i.e., 31 AU/ML); and increased platelet count (401,000 lac/cumm–502,000 lac/cumm) were reported. Radiological investigations showed normal X-ray of dorsolumbar spine and abnormal ultrasonography of the abdomen and pelvis indicative of left ovarian simple cyst with dilated vascular channels in the pelvis.
Medical management of the patient was directed toward use of corticosteroid such as various combinations of antibiotics, analgesics, and antacids; combination of pregabalin and multivitamin. Injectable cyclophosphamide, ondansetron, methotrexate, and mesna were used to manage acute relapses.
Physiotherapy assessment of the patient suggested weakness in proximal muscles of all four limbs affecting lower limbs more than upper limbs and right side more than left side of the body. Superficial and deep sensations were impaired in the right side of the body. Mobility, handicap, and quality of life (QoL) were evaluated using clinical mobility scale, environmental status scale, and WHO QoL-BREF outcome measures.,, The initial, 1 month, and 3 month follow-uP values are given in [Table 1]. Balance impairments were not significant in the patient.
|Table 1: Outcome evaluation at Initial visit, 1 and 3 months follow-up for the patient|
Click here to view
Physiotherapy is directed toward developing good voluntary control in all four limbs, functional balance, and gait training with more focus on functional rehabilitation. The patient is currently under strengthening regimen for all four limbs and functional training for activities of daily living. The patient is currently doing all her daily life activities along with running a beauty parlor and is able to deal with her personal and social responsibilities well. As [Table 1] shows, the improvement in patient's condition can be seen in the objective findings of the outcome measures. [Figure 1] shows the current functional status and level of handicap of the patient. Improvement in cardiopulmonary endurance is a long-term goal for maintaining longer and quality life for the patient.
| Discussion|| |
The patient represents a rare case of GPA affecting the nervous system. The patient is selected for the study as proper medical management along with appropriate physiotherapy showed improvement in patient's QoL. There is no current evidence-based standard of care for patients with GPA and no research was found. There are no researches available related to the role of physiotherapy in GPA, which creates a need for more quality researches with emphasis in long-term QoL for patients. The research supports the role of physiotherapy for improving QoL in stroke and mononeuritis patients through interventions aimed at prevention of contractures and deformities; maintenance of strength; control of pain; balance, coordination and functional training; improvement in mobility and movement potential, etc. This can also help in changing the general attitude toward such “incurable” diseases that only maintenance of condition and prevention of complication is option. Such patients can be provided with better QoL even though the condition itself is not curable. It can be inferred from the present study that the patient can be provided a better QoL by combined approach of medicine and physiotherapy in case of GPA.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms including patient's consent for publication of her images and other clinical information in the journal.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Morris DS, Selva D, Dolman PJ. Endonasal dacryocystorhinostomy in Wegener granulomatosis. Arch Ophthalmol 2010;128:1212-4.
Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, et al
. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum 1990;33:1101-7.
Shafiei K, Luther E, Archie M, Gulick J, Fowler MR. Wegener granulomatosis: Case report and brief literature review. J Am Board Fam Pract 2003;16:555-9.
Adu D, Pall A, Luqmani RA, Richards NT, Howie AJ, Emery P, et al
. Controlled trial of pulse versus continuous prednisolone and cyclophosphamide in the treatment of systemic vasculitis. QJM 1997;90:401-9.
Tumiati B, Zuccoli G, Pavone L, Buzio C. ENT Wegener's granulomatosis can hide severe central nervous system involvement. Clin Rheumatol 2005;24:290-3.
Seror R, Mahr A, Ramanoelina J, Pagnoux C, Cohen P, Guillevin L. Central nervous system involvement in Wegener granulomatosis. Medicine (Baltimore) 2006;85:54-65.
Barofsky I, Cohen SJ, Sugarbaker PH. Selecting a quality of life assessment instrument: Standardized tests clinical assessments or custom-designed instruments. Ch 16. p. 239-49.
Stewart G, Kidd D, Thompson AJ. The assessment of handicap: An evaluation of the Environmental Status Scale. Disabil Rehabil 1995;17:312-6.
World Health Organization. WHOQOL User Manual (WHO/MNH/MHP/98.4. Rev. 1). Programme on Mental Health. Division of Mental Health and Prevention of Substance Abuse. Geneva: World Health Organization; 1998.