Nonspecific Interstitial Pneumonia (NSIP)
An idiopathic interstitial pneumonia with better prognosis than IPF, often associated with autoimmune disease.
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What is Nonspecific Interstitial Pneumonia (NSIP)?
Nonspecific interstitial pneumonia (NSIP) is the second most common idiopathic interstitial pneumonia after IPF. It is histologically defined by temporally and spatially uniform interstitial fibrosis or inflammation, in contrast to the heterogeneous pattern of UIP/IPF.
NSIP is divided into cellular (predominant inflammation, better prognosis) and fibrotic (predominant fibrosis, intermediate prognosis) subtypes. A large proportion of NSIP cases occur in the context of connective tissue diseases — particularly systemic sclerosis, polymyositis/dermatomyositis (especially with anti-synthetase antibodies), and rheumatoid arthritis.
Diagnosis is based on HRCT (basal-predominant ground-glass opacities, reticulation, traction bronchiectasis with subpleural sparing), exclusion of UIP and other patterns, and often surgical or transbronchial cryobiopsy when imaging is non-diagnostic.
Symptoms
Risk Factors
When to See a Doctor?
If you experience any of the following symptoms, seek medical attention promptly:
- Persistent dry cough and exertional dyspnea more than 6–8 weeks
- Suspicion of connective tissue disease with respiratory symptoms
- Worsening exercise tolerance and oxygen desaturation
- Already-diagnosed NSIP patient with rapid worsening of dyspnea or cough
Treatment Methods
Which Department to Visit?
You can visit our Göğüs Hastalıkları department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.
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NSIP — Nonspecific Interstitial Pneumonia (Cellular and Fibrotic Subtypes)
Göğüs Hastalıkları
Nonspecific Interstitial Pneumonia (NSIP) is one of the major idiopathic interstitial pneumonias (IIPs) per ATS/ERS 2013 classification, distinct from idiopathic pulmonary fibrosis (IPF/usual interstitial pneumonia UIP). NSIP is characterized histopathologically by temporally and spatially uniform interstitial inflammation and varying degrees of fibrosis WITHOUT the temporal heterogeneity, fibroblastic foci, honeycombing, or architectural distortion of UIP. NSIP is divided into two histopathologic subtypes: (1) Cellular NSIP — predominant interstitial inflammation (lymphoplasmacytic infiltrate) with little or no fibrosis; better prognosis (5-year survival >90 percent); often responsive to immunosuppression; (2) Fibrotic NSIP — predominant interstitial fibrosis with mild inflammation; intermediate prognosis (5-year survival 70–80 percent, between cellular NSIP and IPF); progressive fibrotic course possible. NSIP often secondary to underlying systemic disease — particularly important to identify since treatment may differ: (a) Connective tissue disease (CTD) — most common association; scleroderma (systemic sclerosis — NSIP is most common ILD pattern; mixed connective tissue disease, polymyositis-dermatomyositis, antisynthetase syndrome with anti-Jo-1 or other anti-tRNA synthetase antibodies, Sjögren syndrome, RA); (b) Drug-induced (amiodarone, methotrexate, nitrofurantoin, statins, biologics); (c) Hypersensitivity pneumonitis (chronic form may show NSIP pattern); (d) HIV-associated; (e) Familial pulmonary fibrosis. Approximately one-third of patients have idiopathic NSIP without identified underlying cause. Clinical: insidious onset of progressive dyspnea on exertion, dry cough, fatigue, sometimes chest discomfort; physical examination — bibasilar crackles (Velcro rales), digital clubbing in advanced disease (less common than IPF); systemic features depending on underlying CTD (skin tightness in scleroderma, joint inflammation in RA, dry eyes-mouth in Sjögren, muscle weakness and Gottron papules in dermatomyositis). Diagnosis: high-resolution CT (HRCT — characteristic pattern of bilateral subpleural ground-glass opacities, reticulation, traction bronchiectasis, often with basal predominance and subpleural sparing distinguishing from UIP), pulmonary function tests (restrictive pattern with reduced TLC, FEV1, FVC, and DLCO; ratio FEV1/FVC normal or elevated), serologic workup for connective tissue disease (ANA, ENA panel, anti-Scl-70 for scleroderma, anti-Jo-1 and other anti-synthetase antibodies for antisynthetase syndrome, RF, anti-CCP for RA, MPO/PR3-ANCA), drug history, environmental and occupational exposure history, often surgical lung biopsy or transbronchial cryobiopsy if HRCT not characteristic. Treatment: corticosteroids (prednisolone 0.5–1 mg/kg/day initially, taper based on response) for cellular NSIP and inflammatory component, addition of steroid-sparing immunosuppression (mycophenolate mofetil, azathioprine, cyclophosphamide for severe disease, rituximab for refractory cases) particularly in CTD-NSIP; antifibrotic agent nintedanib (Ofev) approved for progressive fibrotic NSIP and progressive fibrosing ILDs (INBUILD trial); pirfenidone increasingly used; treat underlying CTD; pulmonary rehabilitation; oxygen as needed; lung transplantation for end-stage disease.
Asthma
Göğüs Hastalıkları
Asthma is characterized by wheezing, coughing and shortness of breath attacks; with proper treatment it can be kept under control.
COPD (Chronic Obstructive Pulmonary Disease)
Göğüs Hastalıkları
COPD is an irreversible lung disease characterized by shortness of breath and chronic cough; quitting smoking slows its progression.
Pneumonia
Göğüs Hastalıkları
Pneumonia presents with high fever, cough and shortness of breath; the vast majority recover with appropriate antibiotic treatment.
Tuberculosis (TB)
Göğüs Hastalıkları
Tuberculosis presents with weeks-to-months of cough, fever, and night sweats; early diagnosis and treatment lead to full recovery.
Pleural Effusion
Göğüs Hastalıkları
Pleural effusion is the accumulation of excess fluid in the pleural space, resulting from imbalances in fluid production and removal, and represents a manifestation of diverse cardiopulmonary, infectious, and malignant disorders.
Pneumothorax
Göğüs Hastalıkları
Pneumothorax is the presence of air in the pleural space resulting in partial or complete lung collapse, classified as spontaneous (primary/secondary), traumatic, or iatrogenic, with tension pneumothorax representing a life-threatening emergency.
Bronchitis (Acute and Chronic)
Göğüs Hastalıkları
Acute bronchitis is mostly viral and resolves spontaneously, while chronic bronchitis is a smoking-related component of COPD.
Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.